We had a very quiet day…just what we needed. We did a lot of reading, took a nap, and watched Cars and Nemo, and Cars and Nemo, and Car and Nemo…
Nothing new to report today…hooray! Labs remain stable with the exception of her lipase which has been trending up all week, not surprised since her pancreas is enlarged. Lucy remains comfortable on the dialaudid IV, hoping to discuss the weaning process as well as a whole slew of other things with Dr. R when he gets back tomorrow.
We are exhausted! We have been here for two weeks with no real end in sight. As you know, we have a very important trip coming up, in approximately ten days. It was decided that we will wait until Monday to make any decisions about going or postponing our trip. We will go, it’s just matter of when! Make-A-Wish will bend over backwards to make this happen for us…amazing!
Last night Lucy was transfer out of the PICU onto the regular floor, not our usual unit, but one we have been on before. They are working at getting us a room on our usual unit as everyone here knows and understands that familiarity breeds comfort. Some of the nurses from our usual unit came over last night to see our girl, they were so happy to see her with her eyes open. We are so blessed to have the love, care, and concern of so many. We feel so loved here.
In order to get transferred to the floor Lucy’s Dialaudid had to be changed from a syringe pump to a PCA pump-patient controlled analgesia. This is to ensure her safety, as well as ensure that Lucy dose not “share” her meds with anyone else.
Medically we have reached a plateau, Lucy is holding steady. Her blood counts are holding and many of her lab values have remained the same. These are all good signs that the inflammatory process has ceased, now we just need to deal with the repercussions of it. Lucy’s liver, spleen, gallbladder, and pancreas are still very swollen. She is unable to sit up at all or even move on her own. We have to physically roll her from one side to the other. She is very protective of her abdomen and is compensating her movements to adjust for pain and discomfort. We decided to hold off on repeating an abdominal ultrasound for a few days because it is very obvious on exam that things have not changed much from her last ultrasound a few days ago. Why do it if you are not going to do anything different with the information.
Lucy’s pain is still under control. She is requiring very few PRN doses of dialaudid. We think that the clonidine patch is helping to alleviate some of her pain thus taking away the need for PRN doses of dialaudid. We are going to increase Lucy’s dose of clonidine in hopes of seeing more improvements with her high blood pressures. They have come down slightly, but not nearly where they should be for a kiddo Lucy’s age.
We are starting to talk about the weaning off process. Pain management came in and basically said that it is a process that takes time, how much time they could not say, it is very individual. Lucy is on a very high does of dialaudid so we are looking into other meds that we can use to step her down gradually. For today, we keep where she is at.
Thank you for this day!
Lucy is s-l-o-w-l-y starting to feel better. She slept most of the night and woke feeling more like herself. Better yet, she is stable enough to be moved back to our regular floor. I will update when we get a room.
Lucy is finally sleeping after being awake for nearly 40 hours, unbelievable! Surprisingly, for being awake for as long as she was, she was pleasant and interactive when you would engage her. She is experiencing episodes of break through pain that suddenly wake her screaming and send her heart rates soaring…poor baby. We are going to have to go up on her dosing to keep her comfortable and hopefully sleeping. I am praying for sleep, peace, and comfort for our little one tonight as her body is fighting so hard for everything.
We started Lucy on Clonidine today with hopes of helping her sleep and also to lower her very high blood pressures. This is a medication that we have been toying with using for some time, but really didn’t want to because we were worried about it bottoming out Lucy’s blood pressures, we would welcome a drop a blood pressure now. We are using the clonidine patch which is a great alternative way of administering a med for Lucy, if it works, since her meds either have to be given IV or rectally, a patch makes life much easier.
I was able to get away for thirty minutes this evening to have a massage in the family resource center. This is a service that the hospital is offering to families for a very reasonable cost. Thirty minutes was all the time I could afford to be away from Lucy, but not nearly enough time to break the surface. Tomorrow Lucy and I are hoping to have a healing touch session, we are looking forward to it.
Last week my in-laws were here with the kids while they were on spring break, it was so nice that they could help us out. This week Uncle Scott, my brother, is here helping us with the kids. This is just the sort of stuff that Disney makes movies out of, uncles with no kids watching his nieces and nephew while their parents are away. We really appreciate his help. Drew, Uncle Scott, and the kids all came for dinner tonight. Apparently, the weather has been beautiful, I wouldn’t know since I have been in a climate controlled environment for weeks. We were able to eat outside in the courtyard. It enjoyed the climate change and just watching my kids run around barefoot.
Thanks as always for your love and support, thoughts and prayers.
When you have as much going on in your body as Lucy does you have to pick and choose what you are going to try and fix and what can wait. Pain control was of the upmost importance so we set out achieve that with the hope that once we achieved it some things would improve for her.
It took several days and did NOT come easily, but I feel that we have achieved a level of pain control that is working for Lucy at the present time. Every 24 hours or so she seems to build up a tolerance and needs more to keep her comfortable. I am hoping that she can manage on the dosing that she is at as we have no room to go up on the drip, but a little room to go up on her PRN doses.
Lucy is experiencing the shakes. Her body temperature is low at around 96.7 degrees, but this is not an unusual body temp for our girl. It is believed that she is experiencing withdrawal symptoms from the ketamine. Oh my gosh, my three year old is going through withdrawal!
Lucy’s body reacts to medications differently. You know the fine print that reads less than 1% of people can experience this, that, or the other…that’s Lucy! Lucy has a history of experiencing paradoxical reactions to medications, this means the opposite effect happens to her. We are seeing this with the dialaudid, she is on an extremely high dose of it, for most of us the dose that she is on would knock us out until Christmas. Not Lucy, in fact she has not slept, in over 24 hours! This is not to say that she isn’t tired, she is EXHAUSTED, she just can’t sleep. There has been mention of trying rectal valium, but it is in the benzodiazepine class of drugs, and guess what…Lucy has paradoxical reactions to all benzodiazepines so we are NOT going to try it. What’s a mama to do?!?
In addition to the insomnia, Lucy’s histamines are firing off and she is constantly itching. We are using dry hospital towels to scratch her face because they are somewhat abrasive and can scratch more surface area all at once. Her Benadryl dose is at it’s max and can only be given every six hours, one dose provides Lucy relief for about an hour maybe two.
All narcotics are constipating, Lucy’s severe dysmotility issues in combination with the side effects of the narcotics are causing issues far beyond what I can express in words. In all honesty, we are not really sure what we can do about this. We can not put anything into her intestinal tract via her g or her j tube, and enemas and suppositories do nothing for Lucy. Abdominal scans have been ordered to see what her bowels look like. No bowel sounds are detected on examination, which simply means her bowels are not moving. This issue in and of itself is huge, but for Lucy it is a part of daily living.
Lucy is in a hyper metabolic state and as a result her body is metabolizing things “differently” than it does “usually”. Lucy is experiencing hyperhidrosis, excessive perspiration. Everyone keeps commenting on her curly hair…she does not have curly hair, it just looks curly because she is covered in sweat. She is soaking through towels, and dare I say she smells a little like the boy’s locker room. I would love to bathe her, however, because she is so sensitive at the moment we really don’t want to do anything to set her off.
Hypersensitive is the state of mind that Lucy is in. Anyone that knows our girl knows that she is particular about how things should go, feel, sound…now all of that is exasperated. Her picking behavior is in overdrive, now that she is awake and more aware she is picking at the stickers at on the side of her crib. we are on the hunt for a new bed for Lucy in hopes of alleviating one issue. At least we can DO something for her.
I am relieved that we have achieved pain control, as is everyone involved in her care! NOBODY wants to go down in Lucy’s dialaudid dosing to see if we can alleviate any of these side effects, for now we need to find ways to make them manageable.
Your thoughts and prayers for continued strength for both Lucy and I are very much appreciated.
Lucy’s eyes have been open and focused on us a lot today! I think that we have achieved a level of pain control that is keeping her comfortable, a continuous infusion of IV Dilaudid and PRN doses every two hours! One of the side effects of narcotics, in particular morphine base narcotics, is intense itching especially of your nose, lips, and eyes. We are using IV Benadryl every six hours to try and control this for Luc. We are also using cold washcloths and anything else that will distract her from itching…ideas anyone?
Speaking of itching, Lucy’s rash is looking better today. There are a lot of theories floating around here as to the cause of Lucy’s rash…I believe that it was a combination of things that caused it, which of course makes treating it all the more challenging. IV fluconazole appears to be helping as well as Aquaphor, IV Lasix to reduce the edema, and another infusion of platelets.
Lucy’s blood pressures continue to remain high despite taking her off the Ketamine. The theory is that the inflammation in her body caused by the MAS has caused inflammation or edema around her heart. Lucy had an echocardiogram and EKG this afternoon to examine her heart. The final report read mild pericardial effusion, which confirms that she has extra fluid around her heart. The thought is that when the inflammatory process in Lucy’s body dies down so too will her hypertension.
I am so relieved that my baby is not in constant pain! I have been told on several occasions that it is going to be challenging to wean her off of the narcotics. We are dealing with today’s problems today and will worry about tomorrow’s tomorrow.
Drew came over after work to see us, as well as Helen. It was difficult to leave the room, but I did when Helen came so that Drew and I could have dinner together outside in the cafeteria courtyard. Umm, when did it turn to summer? It was nice to have some time alone with my husband, I could sense the emotions in both of us tonight. Life is changing…
We are waking to a baby who has her eyes open, laying in her crib, looking very comfortable and at peace. She is SO MUCH more aware this morning than I have seen her in over a week!! Sometime during the night her nasal cannula came untapped from her cheek, we watched as her O2 sats stayed in the mid nineties and decided to leave it off. She has been breathing on her own ever since.
Last night we continued having a difficult time controlling Lucy’s pain, the PICU attending came in and assessed Lucy’s pain at the start of his shift, then about an hour later all h**l broke loose as she was in so much pain that she wanted to crawl out of her own body. Dr. S came back and agreed that she needed to go up on her dosing, he increased her dosing by 30% and moved up her PRN(per requested need) dosing to every two hours vs. every four. I think that we finally have her pain under control, which is good since she is maxed out on her dosing for dialaudid. That’s not to say she has not had a few break through moments, but it is SO MUCH BETTER than it has been!!! We also believe that she has a paradoxical reaction to the drug Nubian, we are discontinuing that med for her.
Lucy’s labs are looking better in some areas and not in others. Her liver numbers are still elevated but are improving. Her triglycerides are in the hundreds not thousands, huge improvement. Today we were actually able to get all of her lab values, unlike the past week. Her blood counts are dropping, she will be getting a platelet transfusion sometime this morning as her counts took a big hit overnight., followed by a round of Lasix. Her albumin, the amount of protein in her blood, dropped again so she will be receiving an infusion of albumin as well. Giving her extra protein should help to draw off some of the excess fluid that she has all over her body as well.
(This picture is from Easter 2010…just remembering.)
I wanted to post earlier but have been busy soothing an cranky, irritable, and agitated baby most of the day. We have been battling pain control. Part of the battle is Lucy’s super high tolerance for narcotics, we are almost maxed out on her dosing. Part of it is not knowing what is causing her pain and trying to find fixes for issues like is she just so itchy that she appears to be in pain but is really in need of being scratched. Lastly, part of it is not having our regular doc here to help us make sense of all of that is going on with Lucy and what we should do about it. We have a bunch of docs working with us but who don’t really know our girly well and that is challenging on oh so many levels.
The Easter bunny found Lucy in the PICU and left her and her siblings some wonderful Easter baskets. On this Easter Sunday we, along with so many, continue to pray for a turn around in Lucy. God is faithful…
After a horrific night it was decided that Lucy needs more long term pain control, the pain management team was called in to consult on Lucy’s situation. Ketamine was used initially because we thought we may only need it on a short term basis, it is not for long term use. Lucy was requiring so much of the med that it created a whole slew of issues from high blood pressures to nightmares/hallucinations. She was put on a blood pressure medicine to control her blood pressures which continue to remain high despite taking her off the ketamine.
After meeting and discussing Lucy with the PICU attending and pain management doctor it was decided that Lucy needs to be put on narcotic pain relief, specifically we put Lucy on Dialudid. It is ten times stronger than morphine yet not as strong as fentanyl. If we put Lucy on fentanyl that also means that she would have to go on a ventilator to help her breath. The fear with putting Lucy on a ventilator is that she will become reliant on the vent for breathing and never come off of it…this is a very scary reality. Dialudid is a very potent narcotic that Lucy has already proven she needs A LOT of to keep her comfortable. If her need continues to rise we are fearful that her respiratory status will change and she would need to be intubated and put a ventilator.
Please pray for peace and comfort for our little one as she is struggling to find it. She is very swollen and itchy. We started Lucy on IV Dialudid for pain, IV hydralazine for her high blood pressures, IV Lasix to help with the swelling, IV Benadryl to help with the itching, IV Nystatin to control any fungus that we think is forming from the rash on her body, and IV Nubian to help with the pain and itching as well, in addition to the other five other IV meds she was already on. Her nurses are really going to be earing their pay today.
I am tired as I have been awake for days…she is finally sleeping and so must I.
There is a lot going on with Lucy and I am going to do my best to try to summarize things.
Lucy’s body is complicated due to the fact that she has mitochondrial disease, a disease that can affect everything in the body down to the cellular level, as a result she has a lot going on with her systemically. A person with mitochondrial disease is at “highest risk for neurological and organ damage during and for the two weeks following an illness. Therefore even a simple flu or cold virus can have devastating effects on the patient, even death." (UMDF)
Lucy’s body is stressed…how’s that for stating it simply. The flu virus has proven to be too much for our little one’s body and is reeking havoc on many of her major organ systems. Pick an organ, any major organ, and I could tell you something that is going wrong with it at the present time in Lucy’s body. Our biggest issues at the moment are being caused by the fact that Lucy has macrophage activation syndrome (MAS), an inflammatory response of her immune system. This syndrome is extremely complex to try and explain, even the doctors who are treating Lucy are having a difficult time understanding and explaining it. Dr. R told us that you will not find a lot of literature out there that explains MAS and mito, saying that he has only found three case studies in I believe Alberta, Canada that describe MAS and mito. All three patients had failing bowels, just like the two patients that Dr. R has ever seen this happen to. Lucy’s immune system had a hypersensitive response to the influenza virus causing her body to send out uncontrolled activation and proliferation of macrophages.
Lucy’s liver,spleen, and gallbladder are taking up a majority of space in her abdomen, as well as her bladder before we straight cathed her and then put in a Foley catheter. Our PICU doctor (who by the way credits himself with saving Lucy’s life in October when she was in need of life saving central access, he likes to remind Dr. R of that often) told us that her liver and spleen are down in her groin area. You can feel it, Lucy’s belly looks and feels like she swallowed a basketball. Her liver is trapping all kinds of red blood cells and platelets making it challenging to get Lucy’s blood counts up. We had to transfuse Lucy with some more packed red blood cells today because her counts went up only minimally with the first transfusion.
Lucy is in respiratory distress because her lungs are so compressed from the extreme swelling of her liver, and having a pneumonia doesn’t help the issue. Her respiratory rates are up as high as 97 breaths per minute. Her breathing is being assisted by using Vapotherm, she is currently at 12 liters of O2 at 45% oxygen to help maintain her oxygen saturations. The high doses of ketamine and morphine that she is on are suppressing her respiratory function as well.
Lucy’s bladder was the size of a small pool on ultrasound, the girl can hold her urine. After voiding close to 200mls of urine, we straight cathed her to see how much residual was left in her bladder. Boy were we surprised when another 250mls of urine came flooding out. We have suspected for some time that Lucy is not emptying her bladder all the way, we are suspicions that she has a neurogenic bladder. All of the narcotics she has been on can also be contributing to this issue. Lucy has a Foley catheter in place which is helping to empty her bladder for her, she is peeing like a champ with the Foley. We are going to follow up with urology sometime in the next week or so about this issue.
Lucy’s gallbladder is inflamed and has increased mucus secretions which is blocking a biliary duct. Surgery was consulted on this issue as well as GI and for the time being we all believe that Lucy’s primary issue is the inflammatory issues related to the MAS which is causing her gallbladder issues. Lucy is on an antibiotic to treat her pneumonia which should cover her gallbladder if it just so happens that there is an infection that is causing the inflammation.
Unfortunately, there is nothing we can do to counteract the MAS, instead we are supporting Lucy’s needs by providing pain control and respiratory support. These types of support come with their own set of problems that I will save for another post as I believe that I have bombarded this post with enough information for one night.
We are trying to keep Lucy comfortable, she is needing more meds to achieve this. Increasing her ketamine dosing has caused her blood pressures to soar, currently 184/114, and her heart rates to plummet into the 90’s from the 160’s. Agitated and irritable would be how I would describe Lucy. She has developed petechia, a very splotchy red rash, all over her body where ever anything comes in contact with an area. This has everyone wondering what her platelet count is. For now we have turned Lucy on her left side in an effort to lower her blood pressure as well as turned down her infusion rate on her ketamine. We will bolus her extra ketamine as needed for pain control. We are investigating other options for pain control.
Lucy is on a continuous IV infusion of Ketamine, Ketamine boluses as needed every hour, and morphine when necessary. The goal is to keep her comfortable. She continues to be on the Vapotherm which is helping her to breath and stay comfortable.
Her blood counts took a huge hit, and as a result we are currently transfusing her with packed red blood cells. I can’t wait to see how pink she will look in the morning.
Thank you so much for all of your thoughts and prayers, love and support!
Lucy’s pain increased tremendously overnight, her heart rates were over 200, and her need for increased oxygen kept growing. I noticed around midnight that Lucy’s body was very swollen especially her abdominal area. At this point she was so uncomfortable that she wanted to crawl out of her own skin. Her pain was uncontrollable. An stat abdominal x-ray was ordered at around 1:30 am only to reveal nothing. I knew this was going to be the case as earlier that day her first abdominal x-ray came back with NO air in her bowel, that in itself is a very unusual finding that had everyone talking about Lucy’s incredibly unique bowels. At this point our nurse and I were begging for someone to call Dr. R or whoever was on call for the DRS team because our girly was extremely distraught. At 4:20 am the resident came in and said that she talked with the DRS doc on call and was told that we could go up slightly in her morphine dosing, but her need for more O2 was growing so we needed to watch her respiratory rate. We gave her more morphine and turned up her oxygen. One of the biggest struggles last night was keeping Lucy’s nasal cannula on, she was bound and determined to take it off. Between the moaning, groaning, and crying out in pain and the constant struggle to keep her from taking off her nasal cannula my heart was breaking for her. Many of the nurses on the floor took turns to help me try and comfort our girl.
At 6:20 this morning the senior resident on for day shift, who knows Lucy really well, came in right away after hearing about Lucy’s struggles. When she arrived things became very critical. Respiratory support was called to Lucy’s bedside as well as ultrasound. She had Dr. R called and told him to come in a.s.a.p. When Dr. R arrived Lucy was in the process of being put on Voapotherm, an oxygen delivering device, to support her breathing. Dr. R immediately knew what was happening to Lucy. He told us that Lucy was having an inflammatory response to the flu virus know as macrophage activation syndrome (MAS). Her liver and spleen were grossly enlarged taking up most of her abdomen causing tremendous pain. Apparently on ultrasound her gallbladder and bladder were also grossly enlarged. Sadly there is nothing we can do to reverse the MAS in Lucy, we can only manage her symptoms and support her medical needs.
In order to support her medical needs Lucy needed to be sedated to control her pain. She also needed respiratory support as a result of being sedated. It was decided that she needed to be transferred to the PICU as her medical needs have increased dramatically.
Lucy is in respiratory distress and is being moved to the PICU ASAP!
Will update as soon as I can
I want so badly to write that things are going better… sadly they are not. Supportive medicine, that is the term that Dr. R is using to describe what we are doing for Lucy. We are trying our best to manage her pain, and let me just say that has NOT been easy. I believe that we have adjusted Lucy’s morphine dosing six times since starting her on it. Most recently we have decreased the duration of time between doses from every three to every two hours. She continues to whimper and moan in pain, it is almost too much for this mama’s heart tonight. Please pray that this change in dosing is what will keep her comfortable tonight as she and I are in need of a good nights sleep.
There was a party of yellow masked and gowned (infectious disease precautions) doctors and nurses in Lucy’s room this morning at 7:20 am, Lucy and I were the only one’s not appropriately dressed for the party. Dr. R came in and discussed her need for oxygen and then proceeded to turn it down only to have half of the room ask him what he was doing. He watched as her numbers quickly plummeted and then said that he wanted a chest and abdominal x-ray ordered bedside. He was reluctant to order an abdominal x-ray because he said that he was not going to replace her j-tube if it was out of position. I agreed to not replacing it today if it were out of position, but rather we could do it later when she is feeling better. He admires my persistence and ordered the abdominal scan too. We also discussed Lucy’s obvious cognitive decline and talked about doing a CT scan of her brain. After talking it over, we agreed that a CT scan was warranted based on her lack of responsiveness.
After getting her chest x-ray we learned that Lucy has a pneumonia, she was started on IV antibiotics this afternoon for treatment. We are not sure if she has an aspiration pneumonia or an acquired pneumonia, so Dr. R put her on an antibiotic to cover both types. The abdominal scan showed that Lucy’s j-tube is in position, good news there for when and if we go back to using it. We stopped enteral feeding a little over two weeks ago in an effort to alleviate the chronic daily pain Lucy was experiencing with feeds. Based on her manometry study results, enteral feeding is going to be challenging, but we are not giving up just yet, just taking a break for a while.
We went down to CT this afternoon, it was quite the ordeal moving Lucy across the hospital with all of her various pumps, monitors, and tubing all the while trying not to agitate her. Soon after her CT scan we received great news, her scan came back NORMAL, thank God! Dr. R told me this morning that Lucy is his sickest patient at the moment, he is worried and shared with me that she is not moving in the direction that he would like to see. I know this, but to hear it from him makes it all the more real.
We want to thank you for all of your thoughts and prayers, love and support. Words can not express how much you have touched our lives. We love hearing from you and share with Lucy all of the prayers being said for her. Times are tough, but we feel so embraced, and for that we are so thankful!
The rash that we are seeing is thought to be broken capillaries caused by all of the edema. The resident called Dr. R to consult with him about Lucy, thank God, and he feels that she is in need of blood product tonight. Lucy’s platelet count is low. How low, we just don’t know because we are having a heck of a time getting any values from her blood because her blood is so full of lipids. Her counts as of Saturday were somewhere in the 50’s which is low. The thought is that she is much lower now because she has had so much going on.
The plan is to get a CBC, type and cross of her blood, and then transfuse her with platelets. The lab just called and said that they need more blood for her type and screen because her blood is not reading correctly in their machines…ugh!
Poor Lucy, she is really struggling. Her body has quickly become dependent on the O2 that we are giving her, the minute she comes off of it her oxygen saturations sink into the 70’s…yikes.
Please pray that she can find some peace and comfort tonight and that she has no reactions from her transfusion.
Things are not going well for Lucy…prayers are very much needed!
Yesterday’s lab work was unable to be processed because there was too much lipid detected in her blood for the machines to get any values. Today we redrew the labs twice, once from her line and another from her arm, only to have similar problems. It turns out that Lucy’s liver is not functioning, she is no longer metabolizing the lipids or fats in her TPN and as a result it is collecting in her blood. Her triglycerides, the lab value that tells you how your liver is metabolizing fat, are astronomically high. For those of you who know about these lab values Lucy’s values are above 17,000 (920 is the high end of normal)! Her blood looks thick and creamy.
Lucy’s electrolyte panel came back with critically low protein, sodium, and calcium levels in addition to just about everything else being off. Lucy is swollen all over, her left eye is swollen shut and her right is nearly there too. Her body is in metabolic crisis. Lucy’s TPN was drastically reformulated this evening and we have taken her off of her lipids. We are infusing her with extra albumin, which is the protein source in her TPN in an effort to get her dangerously low albumin levels back in the normal range. We are hoping that by infusing her extra albumin that her body will start to draw off some of the extra fluid.
Her fevers started climbing to well over 104 degrees this morning and Dr. R decided to give her the Dantrolene to see if that would help lower her fevers. It appears to have reduced her fevers some, she is now consistently in the 102 to 103 range. The major side effect of this medication is extreme hypotonia, in a kiddo with severe hypotonia we now are dealing with having to support her respiratory wise. Lucy is currently on 3 liters of O2 which is helping to bring her pulse ox numbers out of the 80’s and back in the mid to upper 90’s.
In addition to all that I have written, Lucy also has developed a very strange rash under her arms and on her face. We are not sure what is causing this but all agree that if it is a reaction to the Dantrolene it is an awfully late reaction. We are investigating this at the current time.
Pain continues to be an issue, we increased the frequency of her morphine dose but it seems to be wearing off before her next dose is due. We all agree that we need to keep her comfortable, hopefully that means increasing her dosing when need be.
Your thoughts and prayers are very much needed. It is going to be another very long night.
Ugh…fevers, pain, discomfort that pretty much sums it up. Lucy’s fevers are fierce and persistent, sadly there isn’t anything we have given her that helps to reduce her fevers. IV Toradol reeked havoc on her system, causing her to bleed out of too many places. Tylenol has done nothing to bring her fevers down, and because of this and the fact that we have to administer her Tylenol via a rectal suppository, we have decided to stop giving it to her. Lucy’s GI system is completely shut down, both her g and her j are open and draining to gravity,therefore we can not administer any meds via her g or her j ports. Clamping either tube even for a few seconds induces retching which is painful in and of itself, but add a 104 plus fever and body aches and that is just cruel. We are using morphine to control her pain, we have increased her dosing three times and I am going to push for another increase as her heart rates are in the 190’s consistently. We are using cool wash clothes on her body to cool her down, but she is hypersensitive and doing this irritates her and makes her uncomfortable. She will, however, keep a cool cloth across her forehead.
Yesterday Dr. R ordered a series of lab work to check her metabolic function, specifically her lactate level. Unfortunately, we were unable to get any readings on any of her labs both times we drew blood off her line because there were lipids detected in both samples. We did not attempt a third time yesterday as Lucy is already struggling with her blood counts, we will try again this morning. Dr. R is toying with the idea of trying a med on Lucy that is typically given post anesthesia to treat patients who suffer with malignant hyperthermia. The med is called dantrolone, Dr. R is really not sure if it will work to reduce Lucy’s fevers but he thinks it worth a shot. Really it is a way out of the box idea, but with Lucy you need to have someone thinking way out of the box about her.
Praying for some improvement today!
This afternoon Lucy had nasal swabs done to check for any viruses because she continues to run persistent high fevers of 104 plus degrees with no improvement in her symptoms. Less than thirty minutes after the lab received her samples the green sign was added to our door which reads droplet precautions, aka wear a mask if you do not want to be exposed to any airborne pathogens. Lucy tested positive for influenza B, a strain of the flu that I am not sure is included in the flu vaccine. This explains the high high fevers she has been having, extreme pain, and fatigue. Her blood cultures continue to be negative so we stopped the IV antibiotics this afternoon after learning that she has the flu, but are continuing with the IV antifungal until we receive word back from her fungal culture that it is negative. The flu is tough on normal healthy individuals, but in a kiddo like Lucy it has completely and thoroughly wiped her out! Lucy’s immune system once again has not reacted with a very elevated white cell count.
Symptoms wise, not much has changed. Her temperatures remain high. She is retaining fluids which always make her look so different. I think we are going to do a round of lasix to see if we can’t pull off some of those fluids and help her to feel a bit more comfortable. We have increased her morphine dose times three to keep her sleeping heart rates in the 160’s and 170’s. I know those are still some crazy high heart rates, but it beats the 180’s and 190’s. She has not been awake for longer than a few minutes at a time…I miss my girl.
In the midst of all of the chaos we have something we are very much looking forward to doing.
In January while in the midst of crisis, it was mentioned to us that Lucy was eligible for a make a wish referral. I’ll be honest, at first I was so sad to hear the mention of a Make-A-Wish for our girl because in my mind it meant that things were not going well. Let me be even more honest, things are not moving in the direction that we are hoping and praying for. Getting a Make-A-Wish referral is not going to change that, but what it can do is give our family a much deserved break from all of the chaos, to give us an opportunity to celebrate life, and make some memories…memories of a lifetime.
Days before Lucy’s third birthday the Wish Granters came to visit our house and asked Miss Lucy about her wish. She told them she wishes to “see pops…see pinsess!”, translation, she wants to see fireworks and princesses, and what better place to go to see fireworks and princess than… Disney World!
Lucy and our family have been granted a wish trip to Disney World where EVERYTHING will be taken care of for our family, from a limo ride to and from the airport, to our accommodations at Give Kids the World the Make a Wish resort, to theme park tickets and a rental car…simply amazing! We are scheduled to leave on Mother’s day for a week of NO medical appointments, NO hospitalization, NO tests or procedures, and NO school for our big kids!
Jack asked the wish granters when they came why they were doing this for us. We know that Jack is looking for clarity in all that is going on. We think Jack was expecting the wish granters to say something like…your sister is very sick and she deserves this. Instead they answered, “it is hard having a sister who is so sick. Your mom and dad have to give her so much of their time and attention. You all deserve a break and we want to give you that break because we know how hard it can be!” Jack’s face lit up because he knew that this was not just for Lucy but for all of us! I love that Make-A-Wish is making this experience about our entire family and not just Lucy.
Lucy is still running high fevers and cultures are still negative for any growth. Nobody is all that surprised because the feeling is that it is fungus and fungus can take a while to grow on a culture. We did send off to the lab separate fugal cultures to see if we can’t get some information back sooner than her blood cultures. We are continuing to treat her as if she has a bacterial line infection as well until we have proof that it is otherwise. This means that we are running two separate IV antibiotics, one to cover gram positive bacteria and the other to cover gram negative bacteria, in addition to the six other med infusions we are running. Seriously, she is running a med infusion just about every hour, thank God for the nursing staff. We also started her on an IV antifungal this afternoon to be proactive in treating the fungus if it is indeed a fungus that is reeking havoc on our little one’s body.
Several hours after starting her on IV antifungals Lucy’s fever came down slightly and she perked up for about thirty minutes. Beth came to visit us this afternoon at exactly the moment Lucy perked up. It was amazing to see our girly sit up for a few seconds and talk to us for a shot period of time, she even asked to watch her favorite movie here at the hospital, Cars. Sadly she has not felt any better since that time, she has either moaned in pain or slept for most of the day.
Just after posting this morning we learned that the IV toradol that we were using to help combat Lucy’s pain and fevers was causing her to have a lot of bleeding. She had blood in her stool, g-tube drainage, blood clots coming out her nose, and even vomited blood on several occasions. All of this blood loss means that her counts took a big hit today, this is not good for our girl who is already anemic and in need of a transfusion. We were talking with Dr. R about transfusing her in the next week or two, but it looks like she may need it sooner.
Drew and the kids came by this evening to have dinner. Drew stayed with Lucy and e-filed our taxes… yeah we still have to worry about the everyday stuff of life all the while fighting for our daughter’s life. The kids and I had dinner together in the cafeteria, for them it’s a treat to eat in the cafeteria, for me it’s a treat just being able to spend time with them. We came back to Lucy’s room after dinner and the kids watched a movie together… well Lucy watched the kids watch a movie for a few minutes and then rolled over and went back to sleep. Drew and I commented on how “routine” this has become to all of us.
Tomorrow my in-laws are coming to help with the kids so that Drew can actually go to work on Monday and I can stay here with Lucy. We appreciate all of the offers of help, meals, thoughts, prayers, love, and support …we feel so blessed!
Lucy had sustained fevers of 105 most of the day and night. Temperatures that high make for one very hot and irritable baby and one worried mama. Rectal Tylenol did nothing to bring down the fevers. IV Toradol seems to be helping a little, but is causing irritation in her stomach lining which is causing some gastric bleeding. We are going to increase her IV Protonix, a med we use to treat her reflux, and switch her to morphine to help with the irritation.
Her GI system is a mess as she is draining a fairly good amount of brown sludge and blood out of both her g and her j tubes. She is uncomfortable, hypersensitive, and does not like it when you have to move her. She is nauseous, retching and gaging, and trying to throw up, but really she can’t because she does not have the muscle tone to do so. We are having to suction her to help clear her airway.
Her diaper area is raw from the acidity of her urine and stool and the constant yeast infections, it has been like this for months, but has now been taken to new heights. We are going to try a new barrier cream on the skin around her diaper area called Ilex. Only problem, I can’t apply the cream just yet because I have to be able to clean her bottom very well so as not to trap any of the acid under the barrier cream. This is just not something she is willing to have done to her at the present time…I don’t blame her!
Cultures have not grown anything…yet. The suspicion is that she is growing a fungus because of the way she is presenting. Fungal infections are notorious for very high fevers. We have been treating Lucy for fungus off and on for the last few months, her last treatment ended about ten days ago, just enough time for it to grow back. Dr. R told us that it can take days for fungus to grow on a culture, so fungal blood cultures are being drawn today in hopes of getting something back sooner, as well as viral nasal swabs just to make sure we are covering all of our bases. We are going to go ahead and start Lucy on IV antifungals because we are so suspicious and she is so sick.
Drew and the kids are going to head over here sometime this afternoon. Megan and Jack are doing better, Jack is back to his usual but Megan is still so tired. Our kids are on spring break this week, go figure. We are working on plans for next week as far as who is going to be coming and taking care of the kids.
That’s right, only two out of the six of us did not get sick this week. If you want to look on the positive side, that’s 1/3 of our family that stayed healthy. When Drew arrived home last night Jack woke up with a fever, how convenient of Jack as Drew was already going to be taking Megan to the ped’s office in the morning. Jack and Megan tested oh ever so faintly positive for strep throat, so two prescriptions for antibiotics were written and hopes for a fever free day for them are in the near future. As for Miss Lucy, fever free is what we are hoping for as well. Despite being on antibiotics for almost 24 hours she continues to have very high fevers of 104 plus degrees. Dr. R is not speculating about anything, but did say he would see us on Monday. Lucy has slept most of the day, everyone who knows our girl is so sad to see her so sick. So far nothing has grown from her cultures, which is not necessarily a good thing. I am tired as I have not slept most of the day or last night. We want to thank everyone for their thoughts and prayers and offers of help. We can’t imagine doing this alone, nor would we want to.
Pain, pain go away…please don’t come another day!!! It has been one long day/night/morning….Lucy’s heart rate when she is sleeping is in the 180’s-190’s, when she is awake I won’t even tell you because it is too scary to write. We are using several pain meds to try and keep her comfortable. Both Lucy’s g-tube and j-tube are attached to drainage. She is pouring out pea green bile, this is not unusual for our girl, but a major red flag for infection. Dr. R came in early this morning and said he would be shocked if this isn’t a line infection. What has us all concerned is that Lucy has been on continuous antibiotics since the middle of February in an effort to keep the infections at bay.
So far nothing is growing, but it’s early.
I will update more later.
On most days I can handle all of “this”, but when she is in pain none of “this” is bearable!
Your thoughts and prayers are appreciated.
We moved up to the floor at or around midnight. Everyone has been on alert as Lucy is showing signs of sepsis. The ER held her until everyone on the floor was ready for her to come up, once we arrived she has been very uncomfortable, retching, shaking, and screaming in pain. The first round of antibiotics were administered in the ER, and now we believe that whatever bacteria Lucy may have growing in her line has spread throughout her bloodstream thus causing her to become septic. Extra meds have been administered so here’s hoping we can get her comfortable enough to sleep. We are in a private room on isolation until we know what bugs we have growing.
Drew went home to sleep so that he can get the kids up in the morning ready for school and the like. I will update more when we know more.
Lucy went from playing outside on the driveway to complaining of belly pain and laying on the couch with a fever of 103.2 all in the course of an hour. She went from good to bad just like that. A quick change of plans were made, bags were packed, calls were made, and we were on our way to the DuPont ER. We are here, labs and cultures have already been drawn, and we are waiting for a bed on our floor upstairs.
Megan was home sick from school today with a fever and sore throat, Drew will take her in to our ped’s office tomorrow for a throat culture. I am feeling a little better which is an improvement over yesterday! A mama just can’t be sick for long!
Your thoughts and prayers are very much needed. My one regret tonight was that we left Megan sleeping and did not tell her what was going on.
We are still here…just not here if you know what I mean. I have been hit with some upper respiratory yuck that has knocked me down, only I can’t stay down because I have four kiddos and a busy life to attend to. It takes A LOT to knock me down…so A LOT is what I’ve got. The yuck that has invaded my body has caused a sinus infection, bronchitis, and oh yeah borderline pneumonia. I went to the doctor this morning and was prescribed an inhaler, antibiotics, and some super duper cough medicine that will knock you out so you can’t cough. This is not only good for me, but for my husband as well as he has had to listen to me cough up a lung or twenty since Sunday.
Megan came home from school not looking or feeling her best, she complained of a sore throat and went to bed. When she woke she had a fever and has been pretty miserable ever since. My guess, she and I are sharing the same germs. Drew is trying to keep the other kids away, but really that is mission impossible. Our wonderful nurse Anne has not only been taking care of Lucy, but me and the rest of the family these past few days. Poor girl is going to think twice before having a big family of her own one day…we love you Anne.
I have so many amazing things I want to share with you, but I am going to save them for another post soon. Prayers for good health all the way around would be appreciated as always, but even more so tonight.
Sometimes results are not always what you want to hear, sometimes you expect them, sometimes you are surprised by your reaction upon hearing what they are, sometimes you wish you never heard them, sometimes your glad you did, sometimes…
On Friday evening we met with Dr. B, Lucy’s GI motility specialist, to go over the results of her antroduodenal manometry study. I won’t drag this post out, I’ll cut right to the chase, the results are not good. They confirmed what Drew and I and her current team of docs already suspected, Lucy’s stomach and small bowel have little to no motility. The study determined that Lucy has intestinal neuropathy, the nerves in her small intestine do not function correctly thus causing her small bowel to have little to no function. Lucy’s stomach and small bowel are unable to function in any way where she would be able to take in and digest nutrients. Even the small amount of feeds (trophic feeds) that we try to feed Lucy so that her bowel wall is very minimally coated with food to keep the bad bacteria from taking over is going to be a struggle. Her results confirmed that she also suffers with reverse peristalsis, or backwards motility. We were not at all surprised by this finding, her body has so clearly demonstrated this by the sheer number of gj-tubes placed in the last few months. The medications that we trialed did nothing to help improve Lucy’s motility, in fact they caused adverse reactions, retching and nausea. The only real stomach contractions that were detected by the study occurred when Lucy was vomiting/retching, there were only a few very weak uncoordinated contractions of her small intestine noted. These findings confirmed Lucy’s diagnosis of chronic intestinal pseudo obstruction (CIPO), although Dr. B did say that she does not present like a classic case of CIPO because her findings are so severe.
Drew and I sat quietly and listened to Dr. B describe what all of the lines and squiggles on the report represented as Lucy snuggled in on my lap complaining of belly pain from sitting in her wheel chair too long. After all of the researching, discussing, praying, and concern given to this study we surprisingly had very little to say after hearing the results. Dr. B was knowledgeable and professional, but really couldn’t recommend anything in his opinion that would improve Lucy’s motility. He told us that based on these study results TPN is our only option. Due to her dependence on TPN and her failing bowels, life threating infections and liver damage are what accompany this option. All of our surgery options are even more questionable as they will not help her motility, but may help to improve her “quality of life”, if the risks don’t outweigh the benefits. He did say that before we consider removing any portion of her intestines, that we should consider colonic manometry testing, this way we may know if it is just part or all of the intestinal system that does not function. Sadly, we feel strongly that we already know the answer to that without subjecting her to further testing. We all agreed that we need to discuss these findings with Lucy’s current team, and define what “quality of life” means for Lucy.
