Provider Meeting

Today started out with a bang. After yesterday's fiasco, I called the patient advocate this morning. She called the doctors and arranged a conference between myself, Dr. Cammock, the resident cardiologist, and several other members that are providing service and care for Rebekah. We all met and discussed Rebekah's care plan. Dr. Cammock apologized for the miscommunication and then for arguing with me about what I saw. We discussed her nutrition after everything else and they still want to fortify or add stuff so we are going to possibly try soy fortifier. Also, Mended Little Hearts came by and left goodies including a very large teddy bear.

Good and Bad

After my post last night, Rebekah had another bradicardia at around 1 AM. Other than the 2 yesterday, she hasn't had any since immediately after her g tube surgery. So, for me, this is a big deal. The on call cardiologist, Dr. Cammock, came in this morning and tried to argue with me about the way she had her heart decelerations. I told her how I saw it happen and she argued that I saw it wrong. She also told me, again,how she wanted to send Rebekah home soon. I told her I did not think it was a good idea because of the episodes yesterday and she said she would review the data (all of it is saved on the computers) and come back. She never came back. That was in the morning. This afternoon, around 4 pm, the care coordinator, the person who orders the home health supplies when a patient is going home, came to get my information and to talk to me about her going home on oxygen tomorrow. I was shocked. I was never spoken to about a set day for discharge and I know that she can't go home on iv Lasix and she gets worse on oral Lasix and they can't send her home on an air blender (machine that blows air into the lungs at whatever O2 % you set it for). I told the lady this and she said I was right so she would go talk to the doctor and come back. She came back and said the doctor did not realize that she couldn't go home on the blender. When I told the coordinator about how Dr. Cammock had been saying Rebekah needs to go home since Monday and how she never listens to me and argues with things I tell her, she referred me to Service Excellence. A girl from Service Excellence who is a patient advocate came down to talk to me, took notes, and then went to talk to Dr. Cammock. Dr. Cammock told the patient advocate that she had no plans to discharge Rebekah until Saturday at the earliest and that she came back to tell me what she found and that I was asleep. I was eating breakfast when she left, so I wasn't asleep. She then sent her resident in here to tell me that the Bradys from yesterday were not a big deal and were nothing to worry about. I was already stressed and mad and super stressed and, did I mention that I was stressed? So, when he said this to me, the mother of this very fragile child, a mother who is scared that her daughter might depart at the slightest mishap, I kind of blew a fuse. I asked him if he had kids. He said yes. I asked if any of them were or had ever been sickly or frail and he said no. Then I told him that until he had been the parent of a sickly or fragile child that he had no right to tell me that anything I deemed important was no big deal and that if I said it was something of concern, then it is. He argued that he was a doctor and I countered that he was a resident and that I didn't care what Dr. Cammock had told him to say. Then I asked what Dr. Walsh (her primary cardiologist) thought and he said he didn't know because he hadn't talked to him. That's when Dr. Cammock came to the door an asked him to come out to talk to him outside before they talked to me. My dad showed up then so I brought him back and filled him in on what he was about to see if Dr. Cammock came back in the room. I decided that I would allow her the chance to apologize for making mistakes and for lying, but when she came in she started off by saying that I had misunderstood. I asked if the care coordinator had also misunderstood and she said yes. I then called her out on having been trying to push Revekah out the door all week and she started trying to back track on what she said and them told me I heard her wrong for 3 days in a row. Then she changed her story to say that what she said wasn't what she meant. At that point, any calm, collectiveness that I may have been holding on to by very thin threads shredded and I told her very clearly, through tears and gritted teeth, that I never wanted to see her near my child again and that I wanted her to leave. She stood there, said ok, then continued to stand and stare at me. I then told her that I wanted her to get out right then and she she almost ran into the wall trying to get out. I have never been so angry in all my life and I have never spoken to another human being the way I spoke to her. My dad said he had never heard anyone speak to a doctor that way and that he had never seen me that mad. I can honestly say that I had a righteous anger I ever there was one. She never listened to anything I said, never took my opinion of my daughter's condition seriously, and then lied about what she had said and done. I cannot believe she was in charge of my daughter's health on any level. I would also say that I am proud that, in retro spect, my behavior in no way encumbered my witness as a Christian because Rebekah was given to me (and Matthew) to care for and that is what I was doing. Her nurse was infuriated that Dr. Cammock was even considering sending her home, so I feel even better knowing that I was not alone in my assessment of her state of health. In conclusion, with the exception of being congested for most of the day, Rebekah had a great day. I had an overall good day with the exception of a stupid cardiologist road bump.

Just one

Well, most of the day, Rebekah has been stable. She had one fairly bad episode at 5:30pm where her O2 stats dropped for about 5-10 minutes. Then at around 7:30pm, she had a pretty significant heart rate deceleration and O2 sat drop, but she recovered from that one with just a little patting and repositioning. She's not ready to go home yet. She did have some special visitors today though. Our pastor and his wife came to visit. Rebekah was very alert and happy throughout their visit :)

Waiting

The waiting game is getting old, but at least there is an end in sight - sort of. The doctors still want to wait to make sure she has time to recover in case she had a virus they didn't check for. So, her cath lab is scheduled for March 5-6. If she gets worse, they will do it sooner. She is still needing help getting air into her lungs, but doesn't need increased oxygen. She has been stable all day, which is good. Her lungs are not any different from Sunday :( So, we continue to wait for her to show us what she needs.

Looking more stable

She seems to have stabilized. The respiratory therapist got into it with the cardiologist because the cardiologist want Rebekah off oxygen and so turned the flow down even though she was getting 100% oxygen while she was hooked up to the wall. The RT said Rebekah needed the flow but could go down on the oxygen and the cardiologist didn't understand how going down in the flow wasn't the same thing so the RT had to explain it to him. To be fair, he is not Rebekah's primary or even the attending (thank you Jesus) - he was a resident. So, the RT got a "blender" which mixes the room air with oxygen and allows them to give her more flow without more oxygen percentage. Right now she is at 28% oxygen (room air is 21%) and her flow 1.75L which helps her get air in her lungs without working so hard to breath. Since the RT did this, she has had no more bad episodes (she had 2 this morning the first of which required lots of help to recover from). The prayers are working, so keep them up please :)

Too cautious

The doctors are so concerned that she might have a viral or bacterial infection even though nothing has grown and all are negative so far that they still have not scheduled a cath lab. They are especially concerned about aspiration pneumonia which, if she had it, would make the cath lab dangerous. While I appreciate their concern and caution, I do not appreciate them ignoring the idea that I might know something about my child thy they do not know. She aspirated at 4days old, so I know how she looked and acted when she aspirated. I know for a fact that she aspirated then because the next day's swallow study proved it. However, she does not sound, look, or act now like she did then, so that makes me feel fairly confident that she did not aspirate but they will not listen to me. She continues to have severe reflux even with a change to a higher dose of meds to prevent it, so she is always at risk for aspiration. Every time they attempt to ween her off the barely there oxygen, she turns grey and drops her stats. Prayers are a serious commodity.

Monday- maybe we'll do something

She had a really good, uneventful night. She was gurgly this morning so they did a little suctioning in her throat. She immediately went to sleep afterwards :) she is still losing weight because of the Lasix. I gave her a bird bath this morning and she seems to feel better. The nurses and the cardiologists are arguing over her oxygen vs O2 stats. The cardiologists want her off oxygen because that will make the pulmonary edema worse and the nurses want her O2 stats in the high 90s because they are trained that it is supposed to be there. They have had several cardiologists tell them even low 80s is alright if it means she's off oxygen but they won't listen. They also don't listen to me very much so I keep having to remind them that I know her best an that they will e doing what I want whether they like it or not.

Tonight

She is currently sleeping peacefully. Her cardiologist team wants her to be off oxygen because she has pulmonary edema and that can make it worse, but she still needs just a whisper (100mL) in order to keep her O2 stats over 80%. They lost the iv in her right foot. They couldn't place one in her right hand because that was where the first iv was that was placed in Boone and it blew on the way here Friday. The left hand blew on the attempt to flush on the way here. The left foot had been poked to bruising from blood draws. They attempted to place on in her head but couldn't so they placed a central line in her neck. She had to be deep suctioned again shortly before 7pm. Matthew and Niya had to go back to home because of school but I am staying here so she has an advocate and keeping Matthew up to date with frequent phone calls, texting, and FaceTime.

She is struggling to breath more today. Her chest x ray showed an enlarged heart and wetter lungs than yesterday so they are putting her back on the iv Lasix. They are feeding her now and she is up to full feeds through her tubie.

Bad episode

We went to eat supper at around 6:45pm. When we left, she was stable. When we came back at around 9pm, she was unresponsive, struggling to breath, very congested and breathing at around 100 respirations per minute. We immediately called a nurse because her nurse was in another room. The nurse came in, assessed her, and called the doctor. As we waited, we took turns holding her and patting her back so that she could work the congestion out. The doctor came and looked at her. She said her lungs sounded ok and she had already had an increase in diuretics today so she didn't want to go up so we asked for a deep suction. She said we could try and called a respiratory therapist. The RT came up (we had her in the NICU) and said he didn't like her respirations or her O2 stats. She set up and that was when we first saw the night nurse assigned to Rebekah who said she had looked like that since she got here at 7pm. We said there was no way and then we explained what was different for her. The RT didn't think she would pull anything out, but said she would try anyway. She went down 2x and pulled out a moderate amount of white stuff each time. She also said she would come back a little later and repeat it if needed. Then her nurse disappeared again. Don't like her very much. Oh well, we get what we want regardless :)

Mystery

So, her blood panels show no signs of infection. The only sign is her elevated white blood cell count. The cardiologist team believes that it is because her body is trying to fight what is making it not feel good - the fluid on the lungs. she has responded well to the diuretics and her lungs are less white and wet looking. Her heart rate is closer to normal and her respiration is still high, but on level with her personal baseline. They still have not fed her through the g tube so she is receiving all fluids and nutrients through the iv. Her O2 stats are still a little low, but the cardio team said they will accept that if she is off oxygen (apparently that will make her heart condition worse). The doctor over the PICU seems determined that she had to have aspirated regardless of what I or the cardiologist say. In rounds she asked "how soon after the feeding did you notice her going downhill?" I told her it was unrelated to a feeding. She insisted that she had gotten worse after throwing up and I corrected her saying that the throwing up was a result of the not feeling good rather than the other way around and she "whatever"ed me! Regardless, the cardiologist is quite sure that it is all because of her heart and the veins and arteries which we agree with. The decision is that they will schedule her for a cath lab as soon as possible to check her heart on the inside. She was just transferred to intermediate.

Into the PICU

Last night, Rebekah started to scream whenever I fed her through the g-tube. She cannot tell me when she does not want to eat because it is all on a schedule and done for her. She has reflux, but she was actually throwing up with almost every feeding. This morning I called the pediatricians office because she was also sneezing and coughing and sounded a little gurgly. She was a little dusky looking too. We didn't get an appointment until 1:40pm. I took her in and the nurse started a pulse ox and it was 71, so she called for help. We were immediately surrounded by 4 other nurses. They checked her o2 saturation in another location and got her some oxygen. The pediatrician saw her and immediately sent us to the local ER while he phoned ahead and arranged for her to be transported to Brenners 1.5hours away. The ER was ready for us and we went straight back to start labs and X-rays. The nurse in her room did a very poor job of putting in an iv. Both I and the nurse who helped commented but it was still done incorrectly. Matthew and Niya showed up at the ER (I had called on my way there to tell him what was going on). Her pediatrician (Dr. Monroe) met us over there to direct the show and inform Brenners with the lab results. Her chest X-ray came back completely white (very bad). She was tachypnic and had tachycardia ( elevated respiration and heart rate). They gave her some strong lasixs. Her blood gas was good and she had a negative RSV test but her white blood cell count was 27000. Brenners transport arrived and we drove to Winston. On the way, the administered antibiotics to start working on the unknown infection. They also drew blood for cultures and had to redo her iv because the first on made her hand swell severely. She was admitted to the PICU and had more blood drawn. She is currently not allowed to eat and is getting all nutrition through the iv. She also started running a low grade fever after being admitted. They will decide what to do further tomorrow morning.

Cardiologist appointment

Rebekah had a heart echo, chest x-ray, and hip ultrasound today. Her echo showed no changes and the chest x-ray still showed haziness. Her cardiologist is going to present her case to the cardiologist board in the morning and then call me with their decision on what should be done and when. She had a hip ultrasound done because she does not have full range of motion of her hips and legs so her pediatrician thought she might have hip dysplasia. Her hip ultrasound was not yet viewed by the orthopedist, but the sonographer said that her hip measurements were perfect. The only unusual thing was a large chunk of fat between the bones. She said that she's only ever seen that when the hips are dislocated, so she couldn't tell me what the doctor would be able to do for that. So, just one more thing to add to the pile. Just to give you an idea of what we are dealing with specialist-wise, she is followed by all of the following: Speech Therapy, Physical Therapy,  occupational therapy, cardiology, nephrology, ENT, audiology, ophthalmolgy, GI, genetics, orthopedics, a nutritionist, the Kids Eat clinic, plastics, and of course her pediatrician. She is also receiving services through the CDSA and the Extended Care Program both of which help me to coordinate her appointments and get as much as possible done at our home. So far, I seem to have been the one coordinating everything, but I'm hoping that as soon as all the evaluations are finished through the CDSA things will change.

Tomorrow

Rebekah has had a good day today. Yesterday, the nutritionist said that she agreed that straight breast milk is best and the calories she is getting should be adequate. Tomorrow we see the cardiologist again and she is going to be getting her hip ultrasound. Can't wait to see what Dr. Walsh says.

CDSA Evaluation

Rebekah was evaluated and accepted into the CDSA program (child development services agency) and has an appointment with the nutritionist in March for a full eval with her. The PT will contact us for an eval appointment and then we will bring in OT and possibly a professional for baby massage.

This weekend

I have been questioning the use of a fortifier in my expressed breast milk from the very beginning. Because she is on fluid restrictions, the nutritionist wanted to increase Rebekah's calories per ounce. At first, while she was still in the NICU, they used HMF and then they transitioned to Enfamil. Both of these are bovine milk based. I had originally told them that I had gone off of all dairy in order to help her reflux and it had worked. Then they did this. I asked about the base for these fortifiers and they led me to believe that it was not cow's milk based. They lied! I just happened to pass by it in an attempt to figure out how to increase the calories in my own breast milk so that I would not have to use the fortifier at home. I was so irate that I immediately talked to the nurse practioner on call and he took her off until the morning. After the second feeding of straight breast milk, she was no longer refluxing. The next morning, they talked to me and suggested Elecare as a fortifier because it was already broken down into amino acids and is hypoallergenic and easier on the tummy. I agreed. By the time we left the hospital the next day she was refluxing at every meal again. I thought perhaps she would adjust and she had gained weight in the 3 weeks. However, seeing her struggle and then refuse the bottle that she seemed to want before made me question whether it was doing more harm than good. I understand, more than most, the nutritional side that their formula say she requires 318.5 cal in a day and that they calculate breast milk to be 20 cal/oz which would equal her getting 266.6 cal per day on her current feeds if she gets straight breast milk. According to her cardiologist, I can take her up on her fluids to where she gets 293.3 cal in a day. Now, what they don't realize is that she expends a great deal of energy refluxing and then throws up around 5-10 mL/feeding which means that for every feeding she gets with the fortifier, she is actually losing 36-72 cal/day. That would take her down to only getting between 246.5-282.5 cal that she keeps on days she gets fortifier. This is not counting the extra expended calories involved in the refluxing process itself. So, I took command and made the executive decision, which as Rebekah's medical advocate I have the right to do, and took her off the fortifier. Once again, no reflux on straight breast milk. Of course, she is still on Prevacid 2x/day which also plays a role. We see a nutritionist, for the first time since leaving the NICU, tomorrow. We will see what she says and hope that she agrees with me.

The first three weeks at home

Rebekah came home on January 29, 2013 weighing just over her birth weight at 6lb 2 oz. She currently weighs 6lb 8.5oz. That is a weight gain of 6.5 oz in 3 weeks at home versus the 3.5 oz gained in the hospital over the 6.5 weeks she was there. That means I am more proficient at helping my baby gain weight at home, which makes me happy. She takes all feedings and medicines through her g tube and we are working on oral feeding. It takes her a long time to get anything out of a bottle because she has to work harder than most to protect her airway due to her underdeveloped epiglottis. She saw her cardiologist on the 7th of February and he said there were really no changes since discharge. She goes back on the 21st of this month. Last week, after we had started increasing her feedings by 5mL/feed (she is on fluid restrictions due to her multiple heart defects), she started having more trouble breathing and became very congested. I immediately returned her feedings to the original and called the cardiologist who prescribed her a lasiks to help get fluid out of her lungs (we knew it was there because she's had it from birth, along with tachypnea). She is currently taking Prevacid 2x/day for her still uncontrolled reflux.

Introduction to Rebekah

      Hi! Thank you for coming to check up on Rebekah. First, I want to give some background on our family and then on Rebekah and why I am blogging about her. My name is Amber. I am currently working on my Master of Divinity degree from an awesome seminary. I quit working when the doctor put me on bed rest and, after Rebekah was born, I decided, with the help of her doctors, that staying at home, except to go to class, was what was best for Rebekah. I also have an eight year old daughter named Niya. She is very bright and loves her family very much.

    When I was 17 weeks pregnant, we had the general anatomy ultrasound. During the ultrasound, the sonographer said, "Oh no, that's not good." When I asked what  she was talking about, she at first said she had not said anything and then told me that I would have to wait until the doctor could talk to me in a couple of days. I waited and the doctor told me that they couldn't find her aortic valve so I needed another ultrasound in 4-6 weeks. Since we were moving to another city, I set the appointment for 4 weeks out. At that ultrasound, they still couldn't find it, so I had another one where they still had trouble, though  now they were also talking about the dilated kidneys. The kidneys, paired with a bright spot in the heart, are markers for Downs Syndrome, a diagnosis I would have been fine with since I have worked with many special needs children in the school system. However, when they referred me to a place for a level 2 ultrasound, I was told by the cardiologist there that he did not think she had Downs, however she did have what is known as coarctation of the aorta and a small left side of the heart. When present in females, this usually means Turner's Syndrome, another diagnosis that I would have been fine with since it has almost no immediate side effects except for the heart defects. That was when I was 28 weeks pregnant. At 33 weeks, I went into preterm labor and it was stopped, I was given steroid shots for her lungs, and then I was put on full bed rest. At 36 weeks, I saw the cardiologist again and he confirmed his original diagnosis and I was told that she would probably have to have surgery at 2-4 days old. At 37 weeks, she failed a non-stress test because her heart rate had no  variability. They watched me for a few hours and sent me home. 2 days later, the non-stress test not only showed no positive variability, but showed decelerations. They sent me to the hospital and I was induced. She was born at 5:07am on December 14, 2012 weighing 5 lb 14.5 oz. She could breath on her own, but was immediately taken by the NICU team and transferred to Brenners Children Hospital to the PICU (where they send cardiac surgery candidates). Her first echo revealed multiple heart defects including mild hypoplastic left heart structures: mild hypoplastic aortic valve annulus, low normal transverse arch, mild hypoplastic mitral valve, moderate atrial septal defect, moderate left to right atrial shunt, large PDA, mildly decreased left ventricle apex, multiple small mid to apical muscular VSD's with bidirectional bloodflow, dilated right ventricle, moderate LPA branch stenosis, and dysplastic pulmonic valve with significant restricted mobility and moderate to high stenosis. Over the next few days routine and other tests were run which revealed that she had a cleft palate involving the hard and soft palate, a pseudocleft lip (it would have been cleft, but it closed in utero leaving a birth mark, low set ears, slightly wide spaced eyes, brachial cleft cysts (these were mentioned by us later on because they were draining), hydronephrosis (severe on left and mild on right), grade IV kidney reflux, anomalies of the optic nerves, severe "regular" reflux, "comfortable" tachypnea (where the fluid on her lungs causes her to breath much faster than normal), and an anterior anus with poor sphincter tone. We found out, when we could start feeding her, that she had an underdeveloped epiglottis that caused her to aspirate about a quarter of everything she ate, so no breast feeding and all expressed breast milk had to be given through an NG tube. The geneticist saw her and told us she thought Rebekah had Wolf-Hirschorn Syndrome which has a low life expectancy. The blood work came back the next day and showed that she has partial Trisomy 15 which is where, in her case, the genes on the 15th chromosome from the q arm at 15.1-26.3(qter) were added to the p arm of her 15th chromosome so that she has 3 copies of 532 genes from the 15th chromosome. What did this tell us? Nothing, because it had never been seen before. There are no recorded cases, anywhere, of her exact duplication. They did parental blood work tests which revealed that I have a very rare translocation of my 15 and 19 chromosomes. However, further testing revealed that my difference and her difference do not seem to be connected, so they are currently doing more testing because it is too big of a coincidence that we both have differences in the 15th chromosome. She was transferred to the NICU at 2 days old because she would not be having heart surgery yet. She spent 46 days in the NICU and came home with a g tube because, even though she learned to compensate and protect her airway, she would get so tired trying to eat and protect her airway that she could not possibly get enough calories eating orally. The journey through the NICU was long. I brought her home on January 29th, 2013. :)

Just born

first time the sisters met

Bath time

After her g tube surgery, she was intibated

All dressed up

Going Home