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:
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.
Heidi & Brian Fish