Heart Cath Scheduled

Alida’s repeat sedated echo and heart cath lab is scheduled for Tuesday 4/22/14 in Milwaukee. We will head down Monday night as we have to be there at 8am on Tuesday. She will be in patient for at least one night afterwards, possibly several. They will then hopefully be able to determine if another surgery is needed, what that procedure will be, and when it needs to be done. The heart cath will give them information such as:
*Measure pressure and blood flow in the heart’s chambers and in the large arteries around the heart
*Measure the oxygen in different parts of the heart
*Examine the valves of the heart
*Examine the functioning and pressures of the shunt

We will post an update as soon as we can, but it may not be until late next week.

Holding Out Hope

Considering all of the long days we have had, and how long today could have been, it actually went quite well. We had everything organized and packed for a possible overnight stay. We got on the road with plenty of time to make it to 12:30pm lab and a 1:00pm appointment. Compared to Alida’s IV pokes, the lab went very smoothly. She didn’t cry when they put the tourniquet on, she didn’t cry when they put the needle in, and she didn’t even cry while the tech fished around in her arm for the vein that kept rolling away. She only cried a little bit when they were all done and took the tourniquet off her arm. She was a champ.

At her appointment we spent some time going over her symptoms the last few weeks; they watched her sats on the pulse ox, took her blood pressure (102 – a tad high, but not bad), and checked her respiratory rate (52, also a tad high). They got her lab results. Her BNP, which is a prime indicator of heart failure, was only 17, which is actually quite good. Anything over 100 is concerning. We did discuss that it could have been much higher on Friday before she was back on the diuretics. We also discussed that you can’t treat a patient just by the numbers, you have to look at the big picture. They did agree that her symptoms are indicative of heart failure. They looked at the X-rays from Friday and her 3/6/14 echo. They didn’t feel like the echo provided them with enough information regarding her pulmonary arteries and the functioning of the heart. These can be difficult to image on an echo.

Given that Alida is doing okay with the night feeds and since restarting diuretics, we agreed there was no reason to admit her today. They feel more information is needed before making any decisions about the next step. The plan is to have a couple other doctors look at the echo and then likely schedule her for a repeat sedated echo and heart cath lab in Milwaukee. The heart cath would give them the best pictures of her heart and what is going on structurally and with pressures and flows. They will call by the end of the week to discuss any additional findings and get the tests scheduled. It will be an overnight stay and we also discussed the possibility of staying a few extra days so they could do some additional close observation of her to try to figure out exactly what’s going on.

After the additional testing, she may stay there for a surgery or they could decide it’s best to wait and continue to let her grow as long as she’s relatively asymptomatic. As with the first surgery, the bigger she is, the better. They did tell us today that there are other surgical options besides just the Glenn. We didn’t discuss them in a lot of detail today. We will once we have a complete picture regarding the functioning of her heart. One of the most exciting pieces of news today was that she could have the Glenn without needing the Fontan. The fact that this is a possibility gives us hope. From our discussions today, another surgery still appears very likely but knowing that the long road of the Fontan and a transplant may not be eminent is very encouraging.

They have much more experience with hypoplastic left heart syndrome. HRHS, which is right heart and what Alida has, is much more rare. Given that and her other complicating factors and abnormal anatomy, she is a challenging case. There aren’t many other patients they can refer to and discuss what did or didn’t work well for them. So we continue on this journey, as always, one day at a time.

Perspective

I have a cousin who knows way too much about Multiple Sclerosis.
I have a friend who knows way too much about leukemia.
That same friend also knows way too much about lung transplants.
Heidi has a friend who knows way too much about infant GI problems.
Heidi has another friend who knows way too much about cancer.
We all know somebody who knows way too much about cancer.
Heidi and I know way too much about premature babies and congenital heart defects.

I wish we couldn’t draw a heart on paper and explain every chamber and function to someone.
I wish we didn’t know how to spell Aldactazide, clonidine, furosemide, and captopril,
I wish “stenotrophamonus” didn’t roll off our tongues as if we were microbiologists.
I wish the front desk receptionists at the Children’s Hospital of Wisconsin didn’t recognize us and know our names.

There’s a strange evolution and maturation process Heidi and I have experienced. The culmination of that maturation is an acceptance of those who don’t understand what we are going through. We’ve come to find joy in the realization that others don’t understand the things we talk about. I’ve read about MS and leukemia and lung transplants, but I’ve never lived any of those realities. I can guarantee that the only people who truly understand what we’ve been living for the past eight months are parents of babies with heart defects. So for all of our friends and family who haven’t lived what we’ve lived, we are happy.

Here’s another important epiphany for us: everybody has the one thing that is causing them the most amount of stress in their life. Instead of being agitated by someone’s seemingly minimal concern in relation to ours, we find ourselves happy for them. Happy that they too don’t have our same stress. Most importantly, we are happy for parents of healthy babies. In fact, we are happy for ourselves everyday for all of the many gifts that each of our two girls possess.

Many people who live the kind of thing we’ve lived make an effort to raise awareness about a specific disease or cause. The truth is we don’t want people to be aware of congenital heart defects in babies or life in the NICU. Anyone who is truly aware of it probably had to live it and we don’t wish that for anyone. Heidi and I do want to educate and support families who have been thrust into this same world we have. We want to help through our writing and by making ourselves available for correspondence. We want to be a resource.

As we head back to Milwaukee to enter the next phase of our journey with our little heart warrior and her twin sister, we are sad. We don’t want Alida to go back into battle. We don’t want to see the inside of some of those hospital rooms. We know, however, this is our fight and we can do it. We go knowing we have an army of supporters helping us stand strong. We feel your love and we are forever grateful.

Unexpected Friday Clinic Visit

We had noticed near the end of the week that Alida was starting to get wheezy. By Friday it was noticeably worse and she was coughing to the point she started gagging. No fever. If it was a cold, it could be serious for her and if it was something related to her heart, we didn’t want to take any chances in case it was serious. I called her pediatrician and the only opening was in 30 minutes, at 3:45pm with a different doctor that wasn’t her primary. Luckily Brian was home so he stayed with Lucie while Alida and I went to Wausau to see Dr. Shepard.

Dr. Shepard confirmed no fever. She looked at her ears and throat and listened to her breathing. I gave her a quick synopsis of Alida’s last couple weeks – increased vomiting and digestive issues, increased irritability and crying, worse sweating and labored breathing, and worse cyanotic (blue) episodes while crying. She said, “I hate to tell you this, but just by looking at her, I think she’s having heart failure.” Heart failure for Alida right now doesn’t mean that her heart is going to stop beating, it means that her shunt is becoming inadequate and her heart isn’t working as effectively as it should be. They did a chest X-ray which confirmed her lungs were very wet and her heart is enlarged. Dr. Shepard consulted with the on call cardiologist in Marshfield, which happened to be Dr. Dietz. Dr. Dietz thought the issues were due to lung disease, not her heart. I disagreed. Either way, I was in agreement with restarting her diuretics to get the fluid off her lungs and help her breath easier. I have had a feeling in my gut for the last couple weeks that Alida’s heart is not doing well. I felt like it was time to make my concerns heard.

On my way home I spoke with the cardiac team in Milwaukee. I spoke with someone I have not dealt with before. Long story short, that person told me she wasn’t going to step on Dr. Dietz’s toes and to call back on Monday after they could look at the X-ray and discuss with Dr. Shepard and Dr. Dietz. I lost it and started crying. After explaining to her how the last several weeks have been and that we feel like no one is listening to us, she agreed that it sounds like Alida is in heart failure. She discussed with Dr. Ghanayem (the CICU director that had us moved back to the CICU from the 8th floor back in December) and they said we need to come to Milwaukee on Tuesday instead of Thursday and we were given Dr. Ghanayem’s personal pager number. Milwaukee also said they would likely put her on the Thursday surgical round table.

Our suspicion that Alida may need additional surgery is appearing more likely. The day of Alida’s first surgery, on 11/27/13, the surgeon, Dr. Mitchell, mentioned to us during an update that given the poor functioning of her right ventricle, she may be a single ventricle candidate. We had no idea what he was talking about. It’s now looking like that may be coming true. In a “simplified” summary, this is what that means:

1. A more accurate diagnosis for her condition is hypoplastic right heart syndrome (HRHS). This is very rare. It means the right ventricle is insufficient in pumping blood to the lungs.
2. Surgery #1: Pulmonary shunt. Alida had this done already (12/1/13). It’s generally done at birth (but she was too small to have it done at birth, so she was 4 months old) and usually lasts 4-8 months until the baby needs another surgery.
3. Surgery #2: Glenn procedure. They disconnect the superior vena cava (the vein that brings all of the blood from the upper body to the heart to be pumped to the lungs to be oxygenated) from the heart and reconnect it directly to the pulmonary artery. Therefore, all of the blood from her upper body bypasses her heart, goes directly to the lungs to be oxygenated, returns to the heart, and is the pumped to the body. This procedure is usually done at 4-8 months old.
4. Surgery 3: The Fontan. The inferior vena cava (vein that brings all of the blood back to the heart from the lower body) is disconnected from the heart and reconnected directly to the pulmonary artery. This is usually done when the child is 3-5 years old.
5. A heart can function this way, as a “single ventricle” for 15-30 years, and then a heart transplant would be necessary.

I tried to find some pictures to illustrate HRHS, the Glenn, and the Fontan, but HRHS is rather rare (HLHS is more common).

We are packing extended stay bags and bringing Lucie with us on Tuesday in case the trip becomes more than a day trip. The diuretics have helped her this weekend. She sounds and looks better. Thank you for all of your thoughts and prayers as we head to Milwaukee on Tuesday.

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Whole Person

We went to Marshfield on Friday 3/28/14. As usual, the roads were bad. Alida couldn’t eat all morning and unlike most babies, this makes her happy rather than fussy. So she was in a fairly good mood until the actual upper GI test. Brian took Lucie out in the hall while I assisted with the test. They injected about 15cc (half ounce) of barium contrast into her G tube, then the assistants and I held her down while the radiologist took pictures of the contrast moving throughout her digestive system. Alida wasn’t super happy, but it was actually quite interesting to be in the room for the test. I got to see her pacemaker, pacer wires, the G tube balloon and her digestive system. The test confirmed there were no issues with the G tube positioning and her anatomy looked fine. However, it did reveal that she has very bad reflux (we had to roll her onto her side several times so she could spit up barium, nasty) and she also empties really slowly from her stomach into her large intestine.

We then discussed with GI. The theory behind her recurrent vomiting was this:

When she was sedated on 3/6/14, it gave her digestive system a “reset” as she didn’t have anything in her system for about a day and a half. Then we started her on pedialyte, then plain breast milk, then fortified breast milk. The break gave her digestive system time to calm down and it took time for her system to get irritated again and the vomiting to restart. The fact that we stopped the Reglan could also be making things worse as it is supposed to help with motility. That medication would help her stomach empty more efficiently. Then when she had the tube change on 3/19/14, it was like the straw that broke the camel’s back. It was enough to get her stomach even more irritated and restart the vomiting on a consistent basis. They recommended restarting her Reglan four times a day and we also increased her Prevacid dose from 1.3ml to 2.5ml twice a day. We are also giving the digestive meds a half hour before her feeds to try to boost their effectiveness.

Despite the changes, we saw no improvement in her vomiting the last week. She would still have large, couch-soaking vomits once or twice a day. The more we watched the pattern and timing of her vomiting, the more we thought it seemed like it was related to the volume of her feeds. We had weaned her off her night feed and switched her from 5 feeds to 4 feeds per day near the end of February/beginning of March. She seemed to be tolerating it just fine until recently. On Tuesday night I vented her G tube a half hour before her 8pm feed; she pushed out 60ml before I stopped it, otherwise she probably would have pushed out more. This means that she still had almost half of her 150ml feed still in her stomach when we were about to start her next feed. So yesterday we reduced her day feed volumes from 150ml to 90ml. She didn’t vomit all day. Last night we tried a continuous night feed (this allows us to get the same total daily volume in her since we reduced her day feed volumes). By 2am she started crying and gagging. Finally we stopped her feed around 3:30am due to the crying and an attempt to avoid vomiting. Today we did her feeds at 110ml. She had a little vomiting but not as much as before. It’s all trial and error to see what works best and causes the least amount of vomiting. It’s not enjoyable force feeding your child this way. She would be much happier if she wasn’t fed as much. Unfortunately heart babies use a lot more calories just to function on a daily basis so gaining weight is a big challenge.

Our big question is why was she tolerating four feeds per day for several weeks just fine and now we are back to night feeds and an intolerance of volume? I have been in contact with the Milwaukee team several times the last week or so. I spoke with them yesterday and asked about this theory: is it possible her digestive problems are a sign that her shunt is becoming inadequate? If the shunt isn’t providing her body with adequate oxygenated blood, won’t that affect her body’s ability to digest food properly? I would think her digestive system needs that oxygen to digest. If you think of being low on oxygen for some other reason, such as high elevation, all of your organs start to shut down. They essentially told me that is a very probable theory. They offered to have us switch our GI care back to Milwaukee. I told them I don’t know if that is necessary, but I do think she needs to be looked at from a wholistic perspective. It doesn’t make sense to have cardiology doing echos and managing her heart meds, while GI does studies, manages her GI meds, and works on feeding. She is a person, not two separate cases. They agreed and said hopefully we will get those answers during our discussion next Thursday.

We are very anxious for Thursday. Between the vomiting, the poor weight gain, the not so great oxygen saturations, and her general demeanor, we are feeling like the chance of another surgery is very possible. From what we have researched, her current shunt is supposed to last babies 4-8 months until they have their next procedure, called the Glenn. I will discuss that procedure and the future implications after next Thursday, if necessary. We are not trying to be pessimistic about the situation, it’s just a reality we are realizing we may have to face. We are trying to prepare ourself for that possibility.

I am having a harder time taking care of the girls by myself. I will write a separate blog about my struggles as a stay at home mom and what a typical day looks like here.

I also will post their 8 month photos and several other great shots from recently. Although Alida seems to be in discomfort quite frequently lately, her smiles are truly heart warming when she shares them.

More to come…

Dear Doctors, We Would Like Some Answers Please

Alida had two great weeks. After her 3/6/14 sedated G tube change, she was doing really well. The vomiting stopped (it was so nice!) and she seemed less irritable and much happier. This allowed us to put her flat on her back with Lucie more often (normally she is on a wedge to help with reflux); we were able to play with her more and work on her motor development more effectively. She was also gaining weight after a several week plateau. Then she had the mic-key G tube button put in during an office appointment on 3/19/14 and everything went backwards. She vomits large volumes again, particularly in the morning. She cries more and turns blue way more often. When we move her or hold her, she often cries and throws up. It’s hard to see her like this after we had a two week taste of her doing really well.

We called GI on Monday and again today. We told them we feel like it has something to do with the new button – somehow it seems to be irritating her stomach. We deflated the balloon in her belly from 5ml to 3ml. That didn’t make a difference. GI keeps telling us the vomiting is a coincidence and there has to be another explanation – illness, not keeping her elevated after a feeding, timing of medications, timing of feeds, frequency of feeds, etc. We weren’t convinced. So we are going to Marshfield tomorrow for some GI X-rays to make sure everything looks okay. Honestly I am not convinced that they will find anything. If the current tube is angled funny and pushing into her stomach in a way that’s irritating to her, I don’t think that is something that will show up on an X-ray. Hopefully I am wrong and there is a simple, quick-fix explanation.

We have also started having some questions and concerns about her heart. Dr. Dietz’s office told us to write her a letter. So we did. We also sent it to Milwaukee today via email. We have an appointment in Milwaukee on 4/10/14 to have her pacemaker interrogated which will be a good opportunity to discuss our concerns. Here is the letter:

3/27/14

Dear Dr. Dietz,

This letter is to discuss some of our questions and concerns with you regarding Alida. As you are aware, Alida does not tolerate clinic appointments well. This letter should assist in communicating with you prior to her appointment on 4/24/14 in order to streamline that appointment as much as possible. In the alternative, given the depth of our questions, we would be happy to meet with you separately without Alida or have a discussion over the phone. Please consult or discuss with Dr. Fish and Milwaukee cardiology as needed in order to address our concerns.

Alida had a plateau in her weight gain in late February to early March. On 2/23/14 she was 5.000kg. She had her G tube change procedure on 3/6/14 when she was sedated. On 3/12/14, she was still only 4.998kg. However, after the procedure on 3/6/14, her vomiting almost completely ceased. She went from vomiting 2-5x/day to once every 5 days. It was a dramatic difference. She began to gain weight again as a result. On 3/19/14, she received the mic-key button during an office visit. That was a Wednesday. By the weekend her vomiting had escalated again. She has been very agitated since. She also has significant crying spells where she sweats, and turns blue. Her oxygen saturations drop into the mid 60′s during these spells. She does recover from them okay, and her saturation levels do return to the mid 80′s. We are currently trying to figure out why she digressed after 3/19/14. Dr. Fish’s office doesn’t seem to think it’s correlated with the tube change, although we are not convinced of that. The vomiting continues to be significant volumes and is again hindering her weight gain and development.

Besides the concerns over her recurrence of the vomiting and slow weight gain, we have some other questions and concerns about her heart.

1. An echo was performed while she was sedated on 3/6/14. You indicated that this is her baseline echo. What about the discharge echo that was done in Milwaukee on approximately 12/23/13? Shouldn’t that echo serve as a baseline and a comparison be done? When compared, what similarities and differences are there in the size of her right ventricle, the functioning of her tricuspid valve, the pressure of her pulmonary arteries, and the condition of her lungs? Has the echo been sent to Milwaukee for their interpretation?

2. There has been essentially no change in her oxygen needs since we came home from Milwaukee on 1/10/14. We have not been able to wean her from the 1/4L. However, you mentioned that her lung disease looked better on the 3/6/14 echo. This presents us a concern to us. Seemingly if her lungs are better, her oxygen needs should be decreasing. As aforementioned, this does not seem to be the case. Our concern then is the function and adequacy of the pulmonary shunt to supply her with adequate pulmonary blood flow as she grows. With how blue she is getting while upset, she is clearly shunting blood across the ASDs. At some point her lungs should be adequately healed and matured enough that she shouldn’t need oxygen. That means the need for oxygen is due to her heart. You mentioned on the phone that her right ventricle may not be big enough until she is five. In our minds, there is question as to how the shunt can adequately provide her sufficient pulmonary blood flow until she’s five, when it already seems to be showing its inadequacy. The improvement in her lungs seems to be countered by her growth and insufficiently oxygenated blood delivered by the shunt. Therefore, what is the plan or what are the options regarding the shunt and her pulmonary blood flow? If her pulmonary shunt becomes inadequate and her right ventricle does not show adequate growth, what next?

3. She is using the ASDs to shunt blood. We understand that those needed to be left open as pop off valves when her significant VSD was repaired. Are there any concerns that the blood flow occurring across the ASDs could be causing other problems with her heart? From our understanding, it is possible that her right ventricle became so muscle bound from working incorrectly to compensate for the VSD. Are there concerns for similar secondary problems from her heart using the ASDs to shunt blood?

4. Is there any benefit of doing X-rays to look at her lungs and possibly her button? Is there any benefit of doing updated blood work? What is the plan for the next echo?

Thank you for your time and concern in helping us address these issues.

Best Regards,

Heidi & Brian Fish