Hallie has been struggling the last week. She's had increasing abdominal pain and by Monday was vomitting. Of course this wreaks havoc with her POTS so her heart was racing and feeling very heavy. We managed it at home until Wednesday morning when I became uncomfortable with how vehemently opposed to eating she was, despite her vomiting subsiding. I brought her to the ER to rule out a bowel obstruction. I thought a quick X-ray and some labs would give me peace of mind that it was just a virus. Silly me.
What most likely started as a virus turned into so much more. A CT scan confirmed there was air in her bowel wall right by her anastemoses where they stitched it back together after her last resection. This is bad, potentially fatal as it means there is something breaking down, allowing air into the wall. If it goes all of the way through the bowel wall and perforated, it would require emergency surgery to save her life. She also spiked a fever in the ER which could mean things were perforating. It could also mean that she had a blood infection and was going septic. So they admitted her.
We've been here three days now on bowel rest (no food or drink) and iv antibiotics. She has improved rapidly. This morning they did an x-Ray and things have improved drastically. Hallie is anxiously awaiting the arrival of her first meal in almost 6 days. They want to give her one more dose of iv antibiotics and then we will be going home this evening. We are very grateful that what started as such a scary condition, improved so quickly so we can get Hallie back to living life to the fullest.
Hallie is doing well for the time being. She was able to attend camp for a week, which was an amazing experience for her. She was able to spend time with other kids that know what its like to be sick all of the time. It was a great boost to her mentally and physically. She was able to set aside being the sick kid and really just be a normal kid for the week.
She started school and has done amazingly well. It is a combination of her being in a more stable place and the school being well prepared to help her. Hallie has been in school for almost 4 weeks now and hasn't missed a day yet. She is typically to at least 10 absences by now. Hallie has set herself a goal that she would like to have fewer than 30 absences this year. She has not had less than 50 so far, so this is a big goal for her. All of her school team and medical team are working together to help her meet this goal.
Of course, nothing is ever 100% in Hallie's world. Now that she is no longer bleeding constantly from the ulcers, we are recognizing that some of her symptoms were not related to the bleeding, as doctors assumed. Her heart continues to race for unexplained reasons. We have discovered there is a relation to when she is changing positions ie. going from lying down to standing up. It is suspected that she has Postural Orthostatic Tachycardia Syndrome (POTS). She goes in a few weeks to see a new cardiologist that specializes in POTS. This is something my sister has, so we have learned from her. One of the best treatments is hyper-hydration. I give Hallie IV fluids once a week. I can usually tell by how she is acting if its time for her fluids. It makes a huge difference. You can literally see her perking up as she gets the fluids. We are hoping this new cardiologist will be able to help us manage the symptoms better.
But overall, we are thrilled with how well Hallie is doing. We've learned to live in the moment and enjoy life for what it is at this very moment in time.
Hallie had her 4th bowel resection on Monday. The surgery itself went really well. It only lasted about 4.5 hours and they were able to find 2 ulcers. They were about 1.5 inches away from each other. The ulcerated tissue went around the entire circumference of the intestine and was narrowing it. They removed about 3" to make sure there was good blood flow on either side of the ulcers. This was more bowel than we hoped to lose, but we are hopeful that she will have enough to function on her own.
Recovery has been rocky. The first day was great. I was surprised at how well she was doing. Then Tuesday night she began to decompensate rapidly. Her oxygen rate kept dropping. They started her on oxygen. We quickly blew threw 2L, 3L, 4L, and all the way to 6L of oxygen. As they were transferring her to the PICU (Pediatric Intensive Care Unit), her oxygen rate was only 76. Her heart rate started soaring up into the 170's and her blood pressure sky rocketed. It was emergent enough that they didn't let us pack our bags. They packed them for us and delivered them down to us later. It was extremely scary. She spiked a fever, so they drew blood cultures and started her on 3 different antibiotics to cover all possibilities. Tylenol brought down the heart rate and blood pressure. They started her on bipap to have positive air pressure going into her lungs at all times. A chest x-ray the next morning showed a spot of pneumonia and sections of her left lung had collapsed. We focused primarily on getting her lungs opened back up. They were confident that with her lungs open, she would be able to clear the pneumonia. By yesterday, she was on a high flow nasal cannula instead of the bipap.
Today has been a different story. Hallie decided she didn't like the oxygen so she kept pushing her cannula on top of her head. Her oxygen levels remained high long enough to finally convince the doctors to start weaning her off of the oxygen. By this afternoon, she was completely off and quite pleased with herself.
Her bowels are still not functioning. She can't begin to eat or drink until her bowels are processing her stomach contents. For now, she has an NG tube that is suctioning out her stomach intermittently. We are waiting for that to clear out before we even think of letting her attempt food. We did start her on TPN tonight. She hasn't had any nutrition since Saturday. She needs it in order to heal properly. Hopefully, it is just a short term thing.
As long as Hallie does well tonight, they are planning on moving her out of the PICU. The plan tomorrow is to start working on getting her epidural out and switching over to iv pain meds. These tend to be more problematic for her since she is allergic to them. Even with Benadryl and Narcan, she is very itchy and blotchy. We need to find the right balance to keep her pain under control without causing too many other problems. Other than that, we are just waiting for her bowels to decide to kick it in gear.
Hallie has had a very busy few months. She has undergone an endoscopy, colonoscopy, and encapsulated endoscopy. She ended up getting admitted prior to the colonoscopy for a day. The pill cam got stuck in her intestines for 9 days. None of the tests went smoothly. Her bleeding from her ulcer has increased, requiring another blood transfusion.
We have exhausted all options to medically treat the ulcer, so now we move forward with the surgical option, which is what we have tried so hard to avoid. On June 16, Hallie will be going in for a bowel resection. Once they have her intestines separated, they will put a scope in to better see the restructured section. There is a possibility of there being more than one ulcer. If that is the case, they will have to do multiple resections. This surgery caries a lot of risks. She has so much scar tissue, her intestines are completely restructured and malrotated, her other organs are in different locations. It makes me sick to think about all of the risks and what my poor girl will have to go through. We have been down this road too many times. This will be her 4th resection and the 7th time they will cut across her abdomen.
The next 5 weeks are going to be focused on getting her as strong as possible for surgery. We are going to see many of her doctors to make sure there is nothing we are missing or should be doing. We are also going to try and fit in as much family time as possible. Hallie knows she is having surgery. She is nervous, she is upset, she is ticked that she is going to miss her summer. We are working with social workers to try and give her as much fun as possible between now and surgery day.
It's 3:08 a.m. and we've been up for an hour. Why? Miss Hallie woke up at 2:00 and thought her brothers should join the party. I was woken up by them fighting and screaming. The bad part is this has been going on for about a month. Not always at 2:00 a.m. but usually before about 4:30. Hallie went from needing 12-14 hours of sleep a night to about 6 hours a month ago. She started having insane amounts of energy.
At first, I thought it was because she just felt good. After about a week, I figured it was something more. But where do you start trying to figure it out with her? I thought maybe the oxygen was helping a bit too much, but she has been needing less and less of it and still needs little sleep. Her heart tests came back great and her palpitations have gotten better. I talked to other Heterotaxy moms and had her magnesium and a few other levels checked since deficiencies can cause insomnia. Everything came back looking better than it has in awhile.
Finally last week, at a routine psychiatry appointment, I was venting my frustrations and the doctor mentioned that it sounded like Hallie was in a mania phase. Yes, that would describe her very well. It can happen anytime you take anti-anxiety or anti-depressants and Hallie is on a fairly strong one. She feels that when Hallie was throwing up from her migraines so much in August and September, it altered how her body was dealing with her anti-anxiety med and threw her into the mania. We have decreased the dose and we have definitely seen a difference, but it is a slow process. Lexapro takes up to a month to build up in her system, so it can takes up to a month to get the higher dose out of her system.
It never ceases to amaze me how something a simple as a migraine, can cause Hallie to go into such a tailspin. We have the migraines under better control by increasing her preventative med and adding a med to treat them at the onset, but here we are over a month after her last one, still dealing with the effects. Another Heterotaxy mom put it best when she said that Hallie walks a tight rope. Yes, she does. Everything is so delicately balanced in her body. One small problem creates a chain affect that can be devastating. We are hopeful that a simple change in her lexapro will help her get back on track, but in the mean time, it makes for one tired and oftentimes crabby mom.
Hallie had her echo done this morning. She fell asleep and slept through most of it which meant they got great images, including her interrupted inferior vena cava, which is always tricky to find. There has been some debate since it hasn't been visualized in year, so it was nice to see it. This defect in itself is not problematic, but can increase the risks of other problems occurring. It is always good to know what problems we need to be on the look out for.
Everything looked good on the echo. There were no structural problems found, other than what we already knew. They hooked her up to the 48 hour holter monitor to see how her heart functions over the next 2 days. Our big concern with this test is she is severely allergic to the adhesive. She welts up and get open wounds filled with pus. We have Benadryl on board and are praying her skin can hold up long enough to get the information we need. We are going to hematology Tuesday and they will be running a lot of labs to look at electrolytes and other metabolic levels to see if she has any deficiencies that might be contributing to the heart problems. Just one of the many joys of Heterotaxy- one problem can trigger an avalanche of other problems.
We are off to family camp at Flying Horse Farms tomorrow. The kids are excited to show dad and I all of the things they did there over the summer and Tucker is just happy to be able go since he's too young to go on his own.
Oxygen has made a huge difference. Hallie has been up since 3:30 and is hyper, bouncing off the walls. I haven't seen her with this much energy in years. I am exhausted just trying to keep up!
Cardiology went well. Her oxygen stats were 100% with and without oxygen. Her heart rate is down to 107, which is much better. Her EKG is normal. We are scheduling an echo and holter monitor to look more closely at the function of her heart. Her cardiologist doesn't feel it is likely to show anything new in regards to her heart.
He does feel that they problem may lie with her abnormal vasculature. Oxygen is also a vasodilator, which means it helps her blood vessels open up to get better oxygen to her organs. He thinks the problem is that her vasculature is not allowing enough blood to get through, which in turn places a large demand on her heart to push more blood. He is going to present Hallie's case at the cardiology conference next week to make sure we aren't missing anything. He is also going to talk the doctor in Cleveland that has been consulting with me. Then he when like to trial her on sildenafil (Viagra). He can't promise that it will help, but he thinks this has the potential of being a miracle drug for Hallie. Wouldn't that be amazing? It has taken me a long time to get her doctors on board with trying this drug, but I think we are finally getting there.
It has been a busy week! We've fit in psychology, rheumatology, pulmonology, and hematology. We are continuing to work on Hallie's PTSD. It is a long, oftentimes discouraging process, but we will continue to work on it. We are having some success with EMDR treatments, so that is where what we are going to focus on.
Rheumatology told us that Hallie's arthritis has really flared. She has inflammation in her wrists and hands especially, but also her knees, elbows, and ankles. If this was any other child, they would put her on immune suppressants to get the arithitis in control, but that is not an option for Hallie with her already compromised immune system. We can't go up much on her anti-inflammatory because of her ongoing bleeding. We were given approval to add 1/4 pill to try and get things under control. Other than that, there isn't much more they can do for her. She has really been hurting and it is so hard to watch. We have used cherry juice with success in the past to help with inflammation, but without Hallie's g-tube, we can't get her to take it. I did find cherry pills today, so I ordered them and hopefully they will help as much as the juice.
Pulmonology is concerned by how susceptible she is to respiratory illnesses, especially pneumonia. She explained that with Hallie's lack of a functional spleen, her Primary Ciliary Dyskinesia, and the decreased blood flow to her right lung, she has a trifecta of risks for pneumonia. Any one of these factors increase her odds, but the three combined put her at high risk of pneumonia. We have tried many things to help prevent Hallie from getting pneumonia, including extra vaccinations and yet she still seems to get it at least once a winter. The concern is that frequent pneumonia is that it can cause long term damage to her lungs. So Miss Hallie will be getting an airway clearance vest. We will use it when she starts coughing to keep mucus from building up in her lungs in hopes that it will prevent infection from forming.
Hematology was today and it did not go smoothly. Hallie's new port did not work at all. Two weeks ago, it wouldn't draw back blood, but we were able to use it for the infusion. Today, they tried 3 different times to access it and every time, it wouldn't flush, but saline oozed out. It was very painful as well. In all of Hallie's ports, this has never happened. It is so frustrating to have this port to give us better access and it not work. She ended up with an iv to get her iron. We are going to give her another two weeks until her next infusion and then try again. If it doesn't work, she may have to go back in to have it looked at or even replaced. The good news is that her hemoglobin was 9.6 up from 7.6 last time. Hopefully, that means that shots I give her 3 times a week are starting to work. I also had a long talk with her doctor about where we go from here. GI is pushing for a bowel resection, but that carries very high risks. She might not survive and if she does, it could mean being listed for a bowel transplant. Best case scenario, it takes years for her to get back to where she is now. And if lack of blood flow is causing her motility issues and ulcer, a resection is just putting a bandaid on the problem. Her hematologist went over all of her specialists with me and told me that I don't mess around. Hallie has all of the big guns for her doctors. He is going to start by calling her cardiologist and move through her other doctors to try and get everyone on board with trying her on the sildenafil to promote better blood flow. She may still end up with a bowel resection, but at least we wouldn't have to look back and wonder. So hopefully, things will start moving in the right direction.
Please pray that Hallie's port will start working. Pray that her doctors and parents will be guided to make the best decisions on her behalf. And most importantly, pray for Hallie that she will be able to physically and emotionally withstand all that comes her way. She is weary. She is tired of doctors and fighting. She wants to remember what childhood should be like for more than a few hours here or there.
Hallie has been in so much pain the last couple of days. She told me tonight that everything inside and outside of her body hurts. Her belly is hurting her the most, though. She told the school nurse that she felt like her staples were pushing through her stomach. It is days like this that make me want to go ahead with a bowel resection to get rid of this awful ulcer. My logical side knows we need to address the blood flow problems first in order to keep another ulcer from forming. It's just hard on the emotional side. I hate waiting and jumping through hoops to even try her on sildenafil (Viagra) to try and increase her blood flow. I hate watching her suffer. She will be having her MRA/MRV of her gut under anesthesia in a few weeks and we are hoping to be able to move forward from there, but there is the possibility of her needing a CT angiogram done before her doctors will prescribe the sildenafil. Lots of prayers and heating packs with even a bit of Tylenol w/ codeine thrown in to try and help. I've switched up her antibiotics for the overgrowth in her intestines in hope that helps. I feel so helpless some days. Please pray for relief for Hallie.
Hallie had her g-tube taken out today. This is HUGE for her! She has had her g-tube for 4 years, since shortly after her last bowel resection. It has helped us give her nutrition, medicine, and fluids. It has been our safety bet and has been used to keep her out of the hospital many, many times. I have been hesitant to have it removed, even though she hasn't used it in almost a year. But I am finally comfortable that Hallie can gain weight and grow at a normal rate without any additional help.
Hallie was less than impressed. She did not want it removed for some reason. They just remove the feeding tube and put gauze over it and wait for it to close on its own. It has already started closing, but is oozing pretty good. I was changing the dressing tonight and she looked at me with pitiful eyes and asked, "what have they done to my poor little body?" We are hoping and praying it closes quickly and doesn't require surgery to stitch it closed.