Is trust a good thing or a bad thing??
I used to believe 100% that it was an amazing thing!! Today, I'm not so sure. Tonight, its bittersweet.
I am in a rocking chair beside Brigitte's bed with a tiny little light so I can see what I am writing. The monitors are all on. Beep, Beep, Beep....the slow, steady hum of her heart. She's stable right now. Lets hope it stays that way.
Today marked day 14 of Brigitte not having a bowel movement. Keep in mind she takes a double dose of adult Miralax, gets an ex-lax square every other day, and also got a few doses of Milk of Mag. We got NOTHING!! Today her doctor decided it was time for a cleanout. Typically this is done inpatient over a few days in the hospital. This time, its being done at home.
This is the email I got today:
We also don’t typically do this at home. But I know you will be with her and know B very well and we are trying to avoid hospitalization more so for B than for your typical patient. Look out for distension, pain, vomiting, in which case we can ‘t do this at home.
I think this makes sense:
(1) Golytely st at 50ml/hour for 2 hours, then 75ml/hour for two hours, and if doing well, go to 100ml/hour. If seems she is barely holding on but not looking worrisome, stay at 75ml/hour. Max is 2L.
(2) chemistry check tomorrow (chem10) to make sure electrolytes are not altered with the treatment
(3) she can have clear fluids by mouth during this time, but best not to have solids or full liquids. She will certainly loose some weight...but that is to be expected.
(3) Hydration 250ml NS three times per week IV.
What does this have to do with trust you say? Well, I am flattered that her doctors trust me so much. It is by far one of the greatest compliments I ever get....aside from those from other parents. I have learned how to do ALL of her care as well, if not better than some of the nurses we have encountered. I know lab values, I can read an EKG, I can deliver meds and suction, I can put her on oxygen, I can give her shots, and infusions. You name it, I can do it. All I have is a HS diploma. I have learned my daughter's conditions and can recite her entire 4 year history much like I can my own social security number.
This is a letter I wrote to a cardiac surgeon in Miami:
Dear Dr. Burke,
My name is Shannon Kaiser. I have a 3year old daughter, Brigitte 8/18/06, who is currently a patient at Children's Hospital Boston. Brigitte's primary diagnosis is Noonan Syndrome, amongst a very long list of other issues. In 11/06 she had pulmonary valve dilation in the cath lab and only had mild relief of the RVOT. On 12/08/06 she had extensive pulmonary valvuloplasty whereby they opened the RVOT and reconstructed the valve with pericardial patches. She also had her main proximal pulmonary artery dilated. After surgery she had a residual gradient with no pulmonary regurgitation in the setting of a very thickened and restrictive right ventricle. This surgery was performed by Dr. Emile Bacha.
Present day she has severe pulmonary regurgitation. The right ventricular function is normal with a moderately dilated right ventricular chamber. She also has an ASD.
Brigitte has had a very extensive medical course. She has MAJOR feeding issues and has had a LADD's procedure for intestinal malrotation 03/07, she has a Gtube 03/07, and has also had a fundoplication 11/07. All of these were performed by Dr. Jay Wilson. As of 09/09 she has had a Broviac CVL placed to initiate TPN due to failure to thrive. Despite being on TPN 3x a week in addition to a very complicated feeding regimen which also consists of 5 60cc bolus' of Enlive Apple Juice daily, and 12 hour continuous overnight feeds of Boost Kids Essentials 1.5 at a rate of 40cc/hr....Brigitte continues to lose weight. In fact, she saw her GI doctor for follow up on 12/18 and was 12.3k and was also seen on 12/30, weighed on the same scale and was down to 11.3k. There is no explanation for this. Her labwork is totally normal. She has been worked up for metabolic issues, thyroid issues, and diabetes. Dr. K(GI) did order a CXR and it was noted that there was change since her previous CXR and was found to have some significant prominence in her pulmonary vasculature.
The reason that I am writing to you is I believe that Brigitte's weight issues are directly being impacted by the insufficiency in her heart. Her respiration's have increased over the last few weeks. Previously she has been in the low-mid 20's and she has been consistent in the mid 30's lately. She is acting pretty normal though. She seems a bit more dusky around her mouth and does get a bit tired after she has been moving around for a bit. She is not losing fluids through diarrhea, nor is she vomiting. Her reflux is completely under control at this point.
I understand that historically children with isolated PS usually do not need intervention until well into their adult years, if at all, but Brigitte has never played by the rules. We have done everything we possibly can to explain away her weight issues. I should add that at this time she doesn't have any oral aversions and will eat by mouth often throughout the day. Every time I consult with another specialist, they all say it "sounds cardiac related." We are on the verge of adding in more TPN to her regimen and I feel as if we are trading one set of problems for another. It doesn't make sense to me to pump her up with nutrition if her heart could be forcing her to burn it all off. Brigitte has enough medical issues, that I don't want to add liver damage to the list due to longterm PN use. My husband and I are well aware of the risks involved in possibly replacing her pulmonary valve and/or closing her ASD. We feel at this time, given the situation, it may be an option that is needed to help take the strain off her heart and allow her to grow.
We have spent well over 300 nights at Children's in the last 3 years. She has had over 25 major surgical procedures, and countless other studies including, triple scopes, MRI's (never a cardiac). She has been worked up for pulmonary lymphangiectasia, celiac disease, hirschprungs disease, amongst other things. She has been severely truamatized and needs to have her ECHO's done typically with Ketamine/Versed. She is extremely fearful of even a simple examination. I share this just so you are aware of how complicated her sweet little life has been, mostly all related to her inability to gain any weight. I also have 3 other children. Brenna is 8, Jake is 7, and Sean is 6. They are all healthy. You may also be interested to know that my father has Hypertrophic Cardiomyopathy and I have Restrictive Cardiomyopathy and Mild Pulmonary Hypertension.
I am hoping that you may be able to help us,or at least give me peace of mind that I have explored every avenue for Brigitte and we are or have done everything possible regarding her cardiac issues. We are very willing to travel to Miami to see you.
Thank you in advance for taking the time to read this. I look forward to hearing from you.
Here is part of his response:
Thanks for the email, I'm sorry to hear Brigitte has had such a difficult course. Your summary is excellent, you may have missed your calling in medicine. I can imagine how traumatic this has been for you and your family. We have three little girls, and it would break my heart to watch one of them go through what you described. If I may, I would like to show your letter to my CICU director, he has a good feel for babies who fail to thrive after heart surgery, and I value his opinion.
While of course it is very flattering to hear things like this from doctors......it puts an unbeliveable amount of pressure on me as well. Kind of like when she was rushed by ambulance a few weeks ago....and I thought she was constipated and dehydrated. Of course as that morning went on, I realized quickly that things were not as simple as I may have thought.....but nonetheless, I should have brought her to the ED that Tuesday night, not Wednesday morning.
I am not blaming myself at all. Looking back, I know I would have done things the same way. But, you can bet your butt the next time she is vomitting up green bile, we will be going in for an eval instead of waiting it out. You just learn to do things differently.
Tonight, I am learning how to do things differently. I am learning how to do a hospital procedure in my house, by myself, with 3 other children who still have to go to football, get homework done, take baths, make lunches for field trips, run to the pharmacy to get medications.......and, maybe eat!! Bryan is working overtime again!!!
It is becoming more and more apparent that her doctors are valuing my need to be at home with my other children, but are also entrusting her life to me as well. That may sound like a no-brainer......but, the pressure is indescribable. At least in the hospital I have nurses checking on her every hour. So if something happens, its not my fault. Tonight, if something happens, it is my fault. How do you live with that for the rest of your life. Again, you try to make quality of life decisions for 3 older children, but put another's at risk....or you make one childs needs more important and sacrifice 3 others. I can't win.
So today just reinforces why I hate Tuesdays. I have a feeling there are going to end up being a few more days of the week I come to despise as well.
“Trust yourself. You know more than you think you do.” Dr. Spock