Barry and I and all three of our sweet babies went to Atlanta this weekend to meet with Dr. Fran Kendall, a mito expert in Atlanta. One of the wonderful things about going to Atlanta is that my brother lives there now! So we had a wonderful visit with him. We got there Sunday afternoon and visited with our old neighbors, then I got to have dinner and an ornament exchange with 3 girls I used to work with. This was an annual exchange that I have missed for the last 5 years, so it was amazing to be back to participate again and to catch up. On Monday and Tuesday Barry worked from the hotel and I took the kids out and about in Atlanta with my sweet brother! On Monday we went hunting the perfect place for him to take pictures of the kids, had a fun lunch together, and drove all over Atlanta. On Tuesday we actually went to take the kids to see Santa, then we rode the Pink Pig at Macy's, then we were off to a graffiti tunnel for a photo shoot! My brother is a pilot for Delta's regional airlines ASA, but I'm trying to talk him into being a part time photographer. I'll be sure to share pics once I have my hands on them. We barely made it back to the hotel to pick up Barry before check out time. We all had lunch together, and then Barry and I and the kids all headed out to Alpharetta to meet the doctor.
She was amazing. We were there from 4pm-6:30pm. She said that although his lab work is not telling us where the problem is, she feels very confident that he does indeed have mitochondrial disease. This is our second mito expert and our second clinical impression that this is indeed what we are dealing with. She was encouraging though and said that even with all the medical drama we have recently had, she would categorize him as a stable mito patient. That is, we are not in a dramatic downward spiral with multiple systems failing. She believes that he has had dysautonomia symptoms since he was young. But that the symptoms became more noticeable and more frequent with his recent mito crash. Since it has been 3 months since his surgery, it is not likely that he will regain back to where he was before surgery with his dysautonomia symptoms. In other words, he will probably always need medication to maintain his blood pressure. But he may still have some improvements and not need as much medication. Only time will tell. We did manage 3 really good things from this visit. First of all we discussed how everyone (including Dr. Kendall) has told us he needs iv hydration at the first sign of a problem to head off a more serious or escalating problem. But I told her how we've been denied iv hydration when he was symptomatic and we were sent to the ER by the specialist here, and how he is being discharged before it is recommended. We also talked about our desire to keep him out of the hospital and away from flu/other germs if he just needs an iv and is not really sick sick. She agreed and is going to establish us with a home health agency that can run iv fluids as needed. Once we find an agency we want to work with, she will give them standing orders for 24 hours of D10 iv if he is vomiting, extremely fatigued, or having additional difficulties maintaining his blood pressure. This way we will know we have immediate access if it is necessary. Should he need longer than 24 hours hydration, he will need to be admitted. But she stressed that most of the time if we catch it early and treat him, 24 hours is long enough. This will be huge. It also gives us a lot more security in sending him back to school, because we know if it causes a crisis, we will be able to get fluids into him. So the 2nd big thing is we have a plan for school. She will get us a letter that clearly says what needs to happen to keep him safe at school. Mainly that he drink constantly and get frequent snacks, be evaluated for a seating support system so he's not spending energy on maintaining his sit, go back for 1 hour initially for a month, then 2 hours for a month, then 3 hours. 3 hours will be our max for this year. He also needs no more than 10 minutes in pe and recess before a mandated snack and drink break. The third big thing is we are going to do a month trial on oxygen at night with a pulse oximeter and see if he has any cognitive or energy improvements. His sleep study showed he is dropping his sats into the high 80's. He is also having 25 arousals and 2 full awakenings per hour all night long. Frequently when he starts to wake up, he holds his breath, which can be normal. But the fact that they are happening so frequently means that they might be significantly impacting him. So we'll do a trial with oxygen and see if we see any improvements. If we do, we'll continue it. If no noticeable improvements we'll just stop it. We were also able to get the Buccal Swab tests for Mito. It will probably take a few months to get these results back. And they are going to do some systemic blood testing with him next week looking at his organ systems functioning (i.e. kidneys, liver, etc.). It will give us a good baseline to have to look and compare in case he gets sick. We'll see Dr. Kendall now every 6 months. We made some changes to his daily supplements too, adding a few things that she thinks might be helpful.
All in all it was a great trip to Atlanta, and I am feeling much better about our plan and our ability to get him fluids when he needs them. If anyone has any experience with home health agencies, I'd love to hear about it. We need to pick someone this week. Thanks so much for all of your love and many prayers!
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