Need Your Support

[thejohnsongang]

Hey everyone. I am having some personal issues dealing with my daughter right now and could use a little support from all my POTS friends. My daughter (16) has POTS, EDS and Von Willebrand and has been having problems with her GI for months now. She is scheduled to have a motility test done on the 30th at John Hopkins Hospital and will be staying overnight for more testing on the 31st. She is having an colonoscopy, endoscopy, and manometry with TTT. Im nervous, scared and it's causing my POTS symptoms to act up like crazy. Anyone had these done? Know what it's like?

She has been experiencing vomiting, nausea, cramping, diarrhea, constipation etc. since Jan. We finally got the doctors to do more testing, now I'm scared to death. She has to have DDAVP prior to her procedures and I'm very nervous about her bleeding. Any advice? I will be off line for a couple of days while we are gone. I'm going to stay with her, but my husband can't get off work. He will be down on Thur. during the day, but Thrusday night and Friday I'm on my own and I'm scared I'm going to have a spell while I'm there with her from all the stress.

HELP....

Edited August 29, 2007 by MightyMouse
fixed spelling/subtitle

[pat57]

I have have colonoscopy and endoscopy, the prep for the colonoscopy is not fun and everybody hates it, but the procedures are not so bad. I wonder about a 16 year old submitting to it tho. I think it will be hard on her from a modesty point of view.

I cannot speak to Ddavp or bleeding. As for your symptoms , if you have a spell- you will be in a safe place. Take one day at a time, if that doesn't work one hour- if that doesn't work take minutes. You will get through it. Does it really matter if you have a spell? I don think so.

Good luck to you both!

[DancingLight]

The only words of wisdom that I can offer you is that I just finished watching a documentary on the children's center at JHU and it made me feel that if I had a sick child that is where I would want to be! I feel like you couldn't ask for your daughter to be in better hands.

Do you know who her doctor will be for the TTT?

Also, it is such a blessing that they have it set up for parents to stay with their children now.

Many folks on the board have had all of those procedures done--so a search for any of them 'colonoscopy', 'endoscopy', etc. might be useful to you.

These are all fairly common procedures and I think your daughter is in excellent hands--I know that can't take away a mother's worry though!

For YOUR worry, do any of the medications in the benzo class help you in a pinch? Like xanax, ativan, or clonazapam? These can be useful in such a situation where your adrenaline is in high gear. Sometimes JUST HAVING the medication on hand can prevent a spell or panic attack b/c you know you have something to help.

If you have a spell, you can just maybe lie with your daughter and feel yucky together!

Best to you and your daughter,

Emily

[thejohnsongang]

I'm not sure who is doing her TTT. I only know that it is at the Hospital at the Blalock bldg. I do love John Hopkins. They saved her live when she was 6. She had went in a coma and our local hospital sent her down to JHH and they dx her same day with severe food allergies call EGID and had her better and home in less than a week. Her CFS doctor now at JHH was the attending on call when we were admitted. He remembered us as soon as we entered his door this past year. He's great. I know Hopkins is the best place around for her and that the doctors are wonderful and know there stuff. My fear is about her being put to sleep and me being alone for most of the time. My husband has always been there for me, gone to every apt he could with me and most of hers. He works at night so it makes it easier to deal with us sickies here during the day. For 7 years now he has been by my side for all of it and I'm scared to deal with it by my self. Not sure how to anymore. He knows just how to talk to me and calm my fears and keep the stress away most of the time. He's a godsend to me. He came to me at just the right time in my life. He is Anna's step dad, but in her eyes he's her only daddy and she loves him to death. She's his little girl and it is driving him crazy that he can't be there through it all. But only 1 parent can stay, and we decided that it would be me....cause this crazy mom can't stand the thought of not staying with her. It was not an easy argument to win with my husband. My fear of having a spell is while she is in having her procedures done. Before and after I can deal with, I think, we'll be in our horizontal positions together. I have a thing for pacing the floor when I'm nervous and it could get me into trouble. I've been fluid and salt loading so far this week trying to prepare for the trip and stay over. I have also been trying to get lots of extra sleep. All for 2 days worth of tests.

That what most people don't understand about our disorders. For some of us it is 1-5 days of prep for us to do most things. We can't just pick up and go. I guess I'm just being a worrisome mother and should just settle down, but I can't. I cry most of the time when I'm thinking of it all. I have never taken anything for my nerves or to calm me down. I haven't even thought about asking my doctor about it. Now here it is 2 days prior and I'll never be able to get an appointment to see him and I know he won't call anything in without seeing me. Oh well...I get to bite the bullet on this on.

Anna and I have already decided that were are going to be bed buddies while she's in. We plan on watching some TV together, sleeping and having some girl talk together. We love to girl talk..most 16 yr olds don't like to with their moms, but our relationship is different, we're friends as well and share our thoughts with each other on everything. We are even taking some craft stuff to try to learn to make bracelets that you finger crochet. Should be interesting.

We leave home Thursday morning and will be home some time late Friday as long as everything goes ok, which I know it will. I've just got the mom jitters. As for the prep for her procedure..she sure is looking forward to it.. How could anyone????? She's staying home from school of course and we have everything ready for her....baby wipes, desitin and lots of paper. But she's worried about accidents. I tried to calm her fears by telling her we would use the garden hose and then just have to buy her new pants... .... she loved that one.

Anyway now that this is a book, I've got to go make Jello for her to eat for the next 24 hrs. Thanks for the thoughts and input...

[pat57]

why don't you call the DR. office and ask about benadryl , the vet recommended it for my dog who has anxiety when we are on vacation. And my daughters Dr.

recommended it pre air flight- for anxiety.

I have no idea what it does to POTS people.

I think you have good cause to be anxious tho. Remember the anxiety is not dangerous, neither is having POTS symptoms in public.

Again,

good luck to you both!

[Rachel]

Hi Nolie,

I hope that all goes well for your daughter (and for you!). I'll be thinking of both of you. Please let us know how things go. And please tell your daughter that I'll be thinking of her and praying for her. I send along my best wishes and big hugs.

That is so nice that you can go with your daughter, stay with her, and offer support. I'm sure it will be a tough time in many ways, but hopefully you can enjoy some good bonding with your "girl talk" nights. That's really cool that you two are so close.

Take care,

Rachel

[juliegee]

Hi Nolie-

My son had the exact same tests done at JHH a few years ago. I was also a nervous wreck. But, of course, everything worked out beautifully and we both survived. I am happy to answer any specific questions you have.

You will be fine. I stayed with Mack alone at the hospital, while my hubby (who did accompany us for this one!) stayed back at the hotel. Mack brought his laptop to watch movies on during his stay. It kept him/us pleasantly distracted. I slept on a window seat/bed in the middle of the winter and FROZE. You won't have that problem:-)

Your daughter will be pretty much confined to bed, except for bathroom visits. You will be given a log to fill out and note every time she eats or drinks something or has any unusual activity. Because of the manometry probe, they will want her to abstain from undue movement and snacking. She will be encouraged to eat several "large" meals during the 24 hours she is there. This better shows how the GI tract behaves during rest and during digestion. Quick note, JHH is fabulous, especially the group of physicians you are seeing; the food is NOT. There is a large cafeteria with many vendors including: Domino's, Subway, Smoothie King, etc. Any food she would like, you will be able to find.

I was pretty nervous about how my son would react when he woke up from anesthesia with the probe up his nose, down his throat. He was surprisingly OK with it. They took a quick X-ray when he woke up to ensure the manometry probe was in the correct position. The worst part was trying to get a bedpan under him in recovery as he was still reacting to his colonoscopy prep. Poor fellow! That lasted a few hours.

Early the next morning, you will accompany your daughter up to the cardiac wing for the TTT. You will not be allowed to stay with her. That was the hardest for me. Mack's BP was about to plunge prior to the test so he was soooooo anxious. He begged me to stay, which I was unable to do. I planted myself outside of that room until another cardiologist dragged me to a nearby waiting room. I was told the test would last an hour and a half unless something happend. I was called to recovery after 45 minutes.

At Hopkins, they do a 45 minute tilt. If a patient hasn't exhibited signs of POTS (in the firdt 10 mins.) or NMH by that time, they inject a drug to simulate a bit of stress, then the test could conceivably last another 45 minutes. The"worst" happened to my son. I will share his experience so that you can reassure your daughter should the question come up. Mack experienced pre-syncope at 35 minutes, AND he vomited as his BP dropped to 40/0. Vomiting was very frightening with the probe in place, but it did NOT come all of the way out (it's very long as it reaches the small bowel). I think Mack was the first to vomit with the antroduodenal probe in place. It did not become dislodged.

A doctor will be nearby, but not in the room for the TTT. The tech very appropriatley layed him back down as his BP dropped. He recovered somewhat and was immediately given several bags of IV fluid before he was brought to me. He was relieved and victorious after surviving all of that. I asked him how it was and he said, "It just felt like a normal day." At that point he was vomiting continuously and passing out 4-5 times a day. She will get her autonomic dysfunction DX immediately in the recovery room.

The TTT will definately be the hardest part for you as you can't be there. But, she is in very good hands. She will be safe. Drink as much as you can, sit as much as possible. It is really a pretty layed back time as you will be waiting around and entertaining her.

Is Dr. Darbari her GI physician? We love him! "John", was the antroduodenal manometry tech. He will spend quite a bit of time with you. He's also wonderful. It will take several weeks for him to read and analyze all of the data. The results from that test will take awhile. I know Dr. Rowe is already in the picture. Another gem. Your daughter is blessed to have such world class care. You both will be in my prayers. Please let me know if you have any questions.

Hugs-

Julie

[Maxine]

I just wanted to send my prayers, and wish you both good luck on your Trip to JHH.

It sounds like your daughter will be in good hands, and she will have the very best care.

I'm sure your daughter will be fine, especially with you being by her side. When you can't be there through some of the testing, it sounds like the medical personal have a lot of understanding and compassion for the patients.

Take Care,

Maxine :0)

[thejohnsongang]

Julie,

I have been trying to send you a personal message for days and your box has been full. I remembered talking to you when Anna was first dx about JHH and their doctors. I Was going to ask you who his Doctors were and if he had his done at JHH. Yes, Anna does have Dr. Darbari for her testing. Her regular GI at JHH is Anthony Guerriero. Not quite sure who is doing her TTT, but I'm very nervous about it. Dr. Rowe did a "make shift" TTT in his office on Anna back in March. He had her lean against the wall at a certain angle for 20 minutes to see what symptoms she had as well has check her BP and HR. In 7 minutes she hit the floor. It scared the crap out of me. She didn't completely pass out all the way...but she was defiantly out of it for a while. Dr. Rowe told me I would love Dr. Darbari. And after hearing from you, I'm sure I will.

It must have been awful for Mack. The prep reaction and the vomiting with the tube in. Anna has a very weak gag reflex. She has been having trouble all morning with her first dose of prep. I even called the hospital to ask what I do if she does throw it up. She is NOT looking forward to these test. The endo she said won't be that bad, but the other 2...she would rather be beaten with a coat hanger. She is a very, very modest girl and doesn't want anyone to see anything. It has been an issue all her life. She has been a little better with Dr. Rowe about showing her stretch marks from the EDS and letting him check her out. She loves him and she feels very safe in his care. That is one remarkable doctor. She remembers the stories from when she was little and he was the Dr. that saved her life by finding the correct dx and treatment for her. That Dr./patient bond is very important to us. I know you and mack feel the same way about him.

Anyway, thanks for sharing your story with me. I will try to send the PM again. If not, it's ok.

Thanks to everyone for their support. It's a godsend to talk to people who understand, get it and can talk with me about it.

[juliegee]

Hi Nolie-

I'll try to figure out how to empty my inbox- sorry. You and your daughter will LOVE Dr. Darbari. He is very understanding and a huge advocate of our kids. He will BELIEVE your daughter and he will believe in her. He was such a godsend after Mack's local specialists told us he had emotional problems causing him to vomit and faint all day. Grrrrrr. Dr. Darbari looked Mack in the eye and said "I believe you don't feel well. I believe you are sick. I am doing these tests to PROVE it and better help you."

From the testing that Dr. Rowe did, it sounds like your daughter has NMH. She may also have POTS. Mack's HR got to 130, but not in the first 10 mins, so technically he wasn't DXed with POTS.

I know that prep is nasty. Mack also had a hard time with it. He did it at a hotel near the hospital. I hope she's drinking the tiny bottle of stuff. Let her get it down at her pace. She doesn't have to chug it and she can mix it with tastier drinks. With her likely autonomic dysfunction, it will be particularly hard as she will be losing lots of fluid and it may exacerbate symptoms. It did for Mack. We had to stay in Baltimore an extra day or so after the testing, just to allow him to recover.

The anesthesiologists at JHH are so good. (As you may already know.) They definately use a "less is more" approach with our kids. Mack was used to oral versed before the IV and the doctor there just let him have a whiff of goofy gas instead. He wanted to minimize the drugs in his system. I guess less anesthesia will help her have a better recovery and tolerate the probe better upon wakening. (No vomiting.) They actually let me stay with Mack until he was sound asleep.

No worries about modesty. Dr. Darbari and the anesthesiologist will be the only ones in the room with her. Then, you/she will not see them again. Dr. Darbari will do the procedures and then basically disappear. Her regular GI or another fellow/resident will do all follow-up while you are there. In the recovery room, while she may be having trouble with additional BM's, it's just female nurses and you. I got Mack's bedpan and pulled curtains around him while he did his business. He was so out of it, we just left the bedpan under him for a few hours.

John, the antroduodenal tech, will spend the most amount of time with you. He will have a long questionare for you to answer. he will spend time going over the log and will give you lots of instructions and support. he will visit periodically both days and he will analyze her data after the test. Dr. Darbari will be the one to call you and give you her DX.

And, I know you're already being careful, but try not to make the TTT seem scary. She will be tightly strapped in place. She will not be hurt if/when she faints. All of her vitals will be carefully monitored. She will be given loads of fluid as she needs it. They are ready for anything and will keep her safe. Just be reassuring and calm. That's the best way to help her.

You both will be in my prayers. Be sure to let us all know how it goes. (If you think of it- Let Dr. Darbari know Mack is much better and back at school full-time!) E-mail me: juliegee at charter.net if you have any questions.

Hugs-

Julie

[juliegee]

Nolie-

Forgive me if I'm boring you with details....I'm hoping all of this info will lessen your anxiety:-) I forgot to mention that Dr. Darbari will meet with you one last time after the procedures are done. They call them a "3 for 1 special" (endo, colonoscopy and antroduodenal manometry) at JHH. I guess the TTT is just a bonus. He will let you know what he saw during the endo and colonoscopy, etc. And, he will call you (or meet with you in person as you're local) to give you the results of the manometry in a few weeks.

Best of luck!

Julie

[Tessa]

Except the Manometry, I have been through all the tests you mention (some of time even twice) and I can assure you that your doctors seem to know exactly what they are doing and therefore, I am quiet sure that they will take all the precautions needed.

The best suggestion for all the tests is not to be nervous. Tell her to think about something nice while doing them and to remember how important they are for her health and wellbeing.

Furthermore, it is very important to know that someone cares about you while you are having the tests done. With your love and care and the doctor?s care, I am sure that she will be fine.

Please, keep us updated,

Love,

Tessa

[thejohnsongang]

Thanks guys....I'm starting to calm down, but won't guarantee how I will feel tomorrow.

Julie, thanks for all the info and I sent you a private message to your regular e-mail address. You have not bored me with any info. I feel a little better now knowing what to expect. Just to update you on Anna's health issues she has EGID, POTS, EDS, Von Willebrand and her GI issues not related to the EGID. That is what this motility study is for. When she was 6, her EGID was in her esophagus, stomach and small intestine. We will also be able to find out from this test if her EGID has gotten better or worse. But even with her tight food restrictions she's still getting sick. They feel it may be the POTS, but could also be the EDS or something else all together. That why the TTT, to check for the pots issue with her GI. She's lucky enough to get the 4 for 1 special

Today has been horrible for her and we still have several hours to go yet. First her prep is making her sick to her stomach causing severe cramping, nausea and vomiting. I've been on the phone several times today with JHH and have been working around it. Second, as the day has progressed so have her POTS symptoms. She's drinking as much as she can....water, Gatorade, juice, Italian ice, chicken broth and tons of jello in her favorite flavors of orange, lime and peach... but she's so dizzy she's stumbling around and bouncing off the wall going down the hall to the bathroom. Third, she's cussing the makers of the prep for the taste and results. She wants to know why they don't have a chocolate flavor Don't we all wish medication tasted like chocolate. The only flavor they have in our area is ginger-lemon...she referred to it as drinking poison. Thank God she only had to drink 2 of the 1.5oz bottles over the course of the whole day.

On top of all of this she has to use her DDAVP in the morning 1 hour prior to her procedure which she does not like. She needs it to stop any bleeding as the Von Willebrand stops her from forming a complete clot. The DDAVP makes her turn bright red, flushed from the increase in BP and makes her feel like she has the flu. She usually sleeps the whole day after using it, so I might be girl talking and watching movies by myself but that's ok...I will just be in bed sleeping with her if I can. I'm confidant in the doctors, I just don't handle stress at all. Anything more than a minor cut or bleed and I freak out. Not during, but after most of the time. But I know this is coming and started to freak out early. My husband said he would stay with me until the last minute when he has to leave to go to work. But he did reassure me that if anything went wrong or if I just plain needed him to call him on his cell and he'd just leave work and let them deal with it. I'm sure I'll be fine, I'm just a worry wort about everything....especially my kids.

Anna has always been my special child. I was a single mom when I had her and she was a very sickly baby due to the EGID. It took us 6 years to get the answer on that one. Her pediatrician was clueless and we got all the wrong answers for years. But over the years we built a bond that is more than just mother/daughter. We are friends and talk to each other about everything. Having Pots has been a challenge for both of us, but has brought us closer together as we understand each others illness. She's my first born and I'm very proud of the person she is maturing into. I'll have to remember to pat myself on the back when I get done typing....My son is a mommy's boy and a very gentle soul. He is 12 and is the most respectful and thoughtful child I have ever seen, even though he is ADHD. He always lays with me, tells me about the girls he likes and gets me fresh drinks just way I like them...tons of ice. He cooks with me and loves to make cookies when I feel up to it. We share a close bond as well, just not the same as Anna. He likes to be more of a loner and play his video games, hang out with friends or shoot his BB gun.

Thanks again for all your thoughts and prayers...We are going to take our digital camera with us...so if I remember to take pictures, I'll post a link for everyone to see us "chillin' out". I will be off line for a couple of days, but as soon as I can I will let everyone know how things turn out.

[thejohnsongang]

Whew....we made it through. We got home late last night. Usually our 1 1/2 -2 hour drive turned into a 3 hour drive due to a fire and 2 lanes were closed down during rush hour traffic. I promised everyone I would write, but just could not get up the energy until today. It was a horrible 2 days and I'm very glad to be home.

We arrived at the hospital at 9:15 and check in. Anna's surgery/tests were to be at 10:45am. Due to a problem with a child in front of us we did not get into surgery until 2pm. Lets just say a very long wait. They did her endoscopy and colonscopy and then put in her mannometry tube. She was done in a little over 2 hours. She even got the blessing of having some of her "stomach juices" collected for the infectious disease department. They are doing some research and asked for willing participants and Anna agreed to help. Anyway, we to recovery for about 1 hour and then to our room. We ended up getting a room on the childrens oncology floor as all the rooms were filled by the time we got ready for one. At least we got some very nice nurses and a private room. The rest of the evening went fine except for the part where she got caught up in all of her tubes/wires and pulled her IV out. Not a good experience, but still workable. I got the privilege to sleep in this chair that folded out into a bed. Lets just say the floor was softer. Then her pump went off every 2 hours and woke us both us and then the nurse came in every 4 hours to check vitals. Not a good nights sleep for either of us. Friday brought on Anna's most worst day thus far. More so than the prep part. She had a lot of problems with her TTT with symptoms. She was so light headed/dizzy etc. She was having a bad day to begin with then as soon as they started the TTT she started throwing up. She did survive, but threw up again towards the end. They then injected adrenaline and that's were everything took a turn for the worse. I wasn't in the room, but it wasn't long before they came and got me. She literary freaked out. Her symptoms were so bad that she was screaming, crying and begging for them to stop the test. They didn't of course and then she was trying to get all the straps off herself. She kept jumping and twitching and the poor thing remembers all of it. I rushed in as soon as I could and she was just curled up in the fetal position shaking and crying uncontrollably. It took me over a half an hour to get her calmed down enough to talk to me. We moved to recovery and I talked to the tech some about what had happened. He said she had experienced signs of being scared, but pushed through the test to get the results they needed. He said he had not seen any results on her TTT that stood out as POTS/NMH but had technically failed the test due to the vomiting. He did say she did show signs of having something due to the reaction she had when they gave her the adrenaline injection. He said she was inconsolable for lack of a better term. He said she clearly became anxious and irritated as well has kept acting like she was falling or trying to catch her self. It took over an hour to get her heart rate to come back down to around 100. They had pushed her up to 158 at one point. She was miserable and I was frankly mad that they didn't stop the test when she had the reaction that she did. They kept her in recovery for about 2 hours and then moved her back to her room where she curled up with her dad and slept for the next 3 hours. They gave her a medication that I can't remember how to spell or pronounce for her stomach and then she had to eat 1 hour after it to see if it helped with her digestion. At 3:30 they came and took her mannomerty tube out and we came home. She took a shower and went to bed and except for getting up at 11:00 today to eat breakfast that is where she still is sleeping. She is pale, tired and feeling horrible. She told me and her father that she would never let them do another TTT no matter what.

We get the results of all of her test in about 2 weeks. We will also be following up with her CFS/POTS doctor. I want to hear his input on her TTT results. I trust him and want to hear his findings as well.

She clearly has signs/symptoms for POTS/NMH, but the tech said her TTT did not show it. If it didn't show it, then what were all her symptoms that are not related to her HR or BP? I loved the doctors we did see while there, but this was the first time they had ever seen Anna. They don't know her, her history or symptoms. Some were wonderful, others (like the TTT tech) are one's I could live with out. We got to meet with Dr. Dabari as well has with her regular GI doctor, a different cardiologist Dr. Brinker, and more than our fair share of GI residents that clearly wanted to meet Anna as she was an unusual case. I think in the course of 4-5 hours on Thursday evening we had at least 6 come to her room to say hello and look at her chart. We did have a wonderful guy named John her took care of her mannometry tube and notes. We spent the most time with him over the 2 days there and Anna really liked him, Chet the TTT tech she clearly did not like.

At least it's all behind us now and we can hopefully get the answers we need.

Thanks for being there for me. It was better for me than I thought it would be. But believe me...there is no place like home...especially for sleep. I did end up in the Wilmer Eye ER at the hospital on Friday. I woke up with my left eye swollen shut and so sore I couldn't touch it. I waited in the ER for 3 hours before I gave up as Anna was getting discharged. The swelling has gone down some, but it is still sore and my vision is a little blurry. I will be waiting until Tuesday if I can to see my PCP about it. If not then I will go to our local ER here. I just want to get some rest and keep my eye on Anna for the weekend. Thanks again for all your thoughts and prayers. Our whole family appreciates it.

[pat57]

I'm so sorry she suffered like that, and so glad its behind you both!

welcome home!!

[doctorguest]

Not to cast a negative light on JHH because it's a fine institution, but I am not sure doing TTT on the second day after colonoscopy, endoscopy, etc. was a wise decision. In reality, TTT doesn't have to be that traumatic if you do it an outpatient basis. Furthermore, it's has been shown that using isoproterenol or nitroglycerin is not necessary to stimulate BP/HR changes in those with autonomic dysfunction because if you have ANS disturbance, it'll show up during a regular tilt in 10-20 min. Also, a large percentage of healthy people will have a drop in BP and an increase in HR if given isoproterenol or nitroglycerin at tilt. I am sorry that your daughter had such a negative TTT experience.

[Mrs. Burschman]

Doctorguest,

That's good to know. I had thought maybe I had an inadequate TTT because they didn't give me any medications. They just tilted me up for at least 10 minutes and watched my heart go pitter-patter.

Amy

[goldicedance]

Your daughter gets a well-deserved hug from me for her ordeal.

Is there a reason why she had the tilt table test right after the endoscopy-colonoscopy?

It would seem to be, based upon my experiences, it took me a few days to recover from the colonoscopy in particular despite the fact that I was hospitalized the day before the procedure to get IV's during the prep for the colonoscopy.

It would seem to me that a having the TT right after the colonoscopy and endoscopy would skew the results. Not only is the patient recovering from the dehydration but also from the twilight anesthesia. I wouldn't want to have those back-to-back.

Your daughter is a real trooper?

[thejohnsongang]

My ttt was real short. Up for 20 minutes then down. That was it. This is the first time I have ever heard of up, down 1 minute....up, down 30 minutes.....up, down 15 minutes with meds.

She had her TT done after her testing as they put a mannometry in place to test to see if POTS?NMH was causing her vomiting or GI problems. She did not have twilight sleep, she was put all the way under for just over 2 hours. She was dehydrated even with IV fluids and had not recouped from her first set of tests. They had to place the mannometry tube surgically, so they did all the "fun" tests first. They even had her fasting for the TT.

Not sure why, but that's how they scheduled it. I awaiting a call/e-mail from her pots doctors to get some answers. I e-mailed him this morning. I want to know why they gave her an injection of medication to raise her HR. Why they didn't stop the test when she freaked out the way she did. And what the test showed besides what I was told in the room. Hopefully I will have some answers soon. When I get them, I'll let you all know.

I will pass on all the hugs and encouragement to her. She feels like she has been put through the ringer and is still sleeping off the effects of her stay at JHH. but she is a trooper and won't stay down for long....Oh how I envy her energy level.

[juliegee]

Oh Nolie,

I've been out of town (funeral in MI) and am glad to be back. I've been so anxious to hear how things went for you guys. I must say that TTT experience sounds like an awful experience. Please pass on gentle hugs to your daughter from me. What poor judgement that tech used in carrying on with the test in light of your daughter's extreme anxiety. I would guess that vomiting with the antroduodenal manometry probe in place would have ended the test...but injecting her with meds after all of her extreme reactions? Unbelievable.

Her whole test sounds like it was done strangely per your quote below:

"My ttt was real short. Up for 20 minutes then down. That was it. This is the first time I have ever heard of up, down 1 minute....up, down 30 minutes.....up, down 15 minutes with meds."

As I mentioned before, Mack's test at JHH was very different. He was down for 5-10 minutes for a baseline check and then up for 45. Good thing as his BP didn't plummet until 35 minutes despite the fact that he was very symptomatic (lightheaded, nauseous, etc.) His vomiting occurred exactly as his BP dropped to 40/?, which is the one-to-one correlation between intestinal GI dysmotility and autonomic dysfunction that they are looking for. I was told that if nothing happened (POTS nor NMH), then Mack would be injected. (Not sure if he would be up or down for the injection) But after the injection (that never happened for him), he was to stand for another 45 minutes.

Your above description doesn't sound anything like their normal protocal. The only thing I can think of that changed everything was her extreme reactions, vomiting, etc. Clearly, they should have stopped things alot sooner. Poor baby. I am very anxious to hear what Dr. Rowe has to say about her experience. My guess is that her test shouldn't be considered valid.

A TTT alone can be very difficult for someone with dysautonomia, but having it done with the manometry probe in place must be excruciating. Mack's test was several yers ago. We were told that he was the 1st to vomit with the manometry probes in place & they stayed in place! (For those who don't know, thiis is a probe that goes into the nose, down the throat, into the stomach and into the small bowel and stays there for 24 hours.) I guess your daugter gets to join that exclusive club:-) We were told that this combo of tests conclusively proved that Mack's motility issues are caused by an autonomic neuropathy. As far as I know, these are the only docs in the world to do the antrodoudenal manometry and TTT at the same time. For us, it provided valuable information that led to a better treatment plan for Mack.

Sounds like it provided a nightmare experience for your daughter. I agree with Doctorguest. The colonoscopy should ideally be doen at another time. It is so traumatic for kids with dysautonomia because of the dehydration issues. The antroduodenal manometry probes are placed while the patient is under general anesthesia (I guess because they are children. I know adults can do it with twilight anesthesia) so the endoscopy really isn't really a substantial additional trauma.

I'm glad you liked Dr. Darbari and John. Both gems!

Big hugs to you both, Nolie. You survived and hopefully will glean some good information form these tests.

Hugs-

Julie

[thejohnsongang]

Julie,

I haven't heard back from Dr. Rowe yet. Not sure if he his getting Anna's results from the TTT together or just out of the office. He usually writes back the same day. She goes to see her pediatrician today. A new one in the same office. This one did her internship under Dr. Rowe and with the Pediatric Allergy Clinic at JHH. Hopefully she will better understand Anna and all of her issues. Dr. Rowe recommended her. Anna is back in school now, but still recovering from all of her tests. She comes home and falls into bed and sleep all afternoon and night. She is slowly regaining her energy. She swears she will never have a TTT done again, and I really don't blame her. I only got to meet with Dr. Debari while I was in the OR with Anna prior to her being put under. Her regular GI spent most of the time with us. Dr. Debari said he remembered Mack and was glad to hear that things were going ok for him. John was a special soul to us during our visit at JHH. Anna really liked him a lot and enjoyed his honesty about what to expect. He also remembered you and Mack and sends his hello's to you.

At least it is all behind us now and we can go forward from here. She just wants to eat and either keep it down or not have pain when she does. I will let you know what Dr. Rowe has to say.

Nolie

[juliegee]

Hi Nolie-

Mack was pretty punk for a few days after all of those tests too. I bet Anna's back to her "normal" physical self pretty soon. Emotionally, I bet she's got a lot to get over. Dr. Rowe's so good about responding. I know you'll hear from him soon. I can't imagine that Anna's TTT will be valid as the tech seemed to do an awful lot of extraneous "up and down" probably in response to Anna's symptoms. I'm really curious about Dr. Rowe's thoughts about the whole debacle.

It's so wonderful to meet another family that's going through the same thing we are/were. I wish we were sharing happier similarities:-) Let us know what you find out.

Hugs-

Julie

[thejohnsongang]

Julie,

I heard back from Dr. Rowe and unfortunately he doesn't have the results of her TTT. He did state the following:

Nolie: the tilt results show no signs of NMH or POTS, but I'm not surprised by this, as she is on treatment, and her lightheadedness is in fact well controlled by the combination of Florinef, Yasmin, and the higher salt intake. The isoproterenol during stage 2 of the tilt test is pretty unpleasant for some people, and can cause shaking and a panicky sensation, so her response is not that atypical. Anyway, the tilt test can't be used in someone on treatment to diagnose NMH or POTS, but it can be helpful in sorting out whether the nausea and vomiting are related to upright posture, so we need to wait for the manometry results. I don't have access to those results.

So we are awaiting the results. I can't believe that I haven't heard anything yet, but I guess since GI is handling it they are waiting for all the results to look at them all together before making a dx.

Anna is starting to feel a little better, but I had to take her to our local pediatrician yesterday. She has a pretty bad sinus infection. Not sure why...hospital, decreased immune system due to fasting/testing or just plain bad luck. They put her on antibiotics 500mg 3 times a day. They always treat her sooner than they would other patients considering all of her health issues. Her spirits are up though and she even had a job interview yesterday for a PT job at Baskin Robins. She starts training next week. I'm worried about her working, but she wants to so bad....so we are going to let her and just watch her to make sure she doesn't overdue herself. She wants to save up to get her own car.... "teenager's" you know how they can be.

And yes, it sure has been nice talking to you as we have gone through the same thing, but I do wish is was on a happier note. Everyone here is great and it has been a wonderful help. Sometimes I feel so isolated in our own community because no one understands.

On the upside (if you want to call it that) when we went to the school to make accommodation's for Anna and to set up her 504, the new Asst. Principal was wonderful. His daughter has POTS so he understood everything and we didn't have to sit for hours to try to explain it. We did sit for hours and talk about local treatments and which one our daughters was on. His daughter sees the same cardiologist that I do. Sometimes it is a small world. Anna went to visit him the next day and she spent some time with him and he assured her that this year she would have a 504. He said it was necessary for collage and couldn't believe that they didn't do it last year. He also said that it was a lot of extra paperwork, but as an educator it was his job to make sure it was in place for her. What a great guy. Anna really liked him and I look for them to build a good friendship over the year. I think he will be a good advocate for Anna when she starts looking into colleges. It always helps to have more than one person in your corner.

Anyway...good news and no news here. I'll keep you posted when I get her results.

Nolie

[Mrs. Burschman]

Nolie,

What a great coincidence! I'm glad you didn't have to explain it!

[juliegee]

Wow!

I wondered about that but didn't get around to asking. They didn't discontinue her meds???? What a traumatic waste of time. The results of the antroduodenasl manometry will actually take a few weeks. John has to go through tons of data, analyzing the entire 24 hours Anna was there and then trying to correlate with her log and activities, i.e. TTT. Dr. Darbari should call you with results. Let us know what you learn.

That's fabulous about Anna's new principal! That's God way of making up to you for that horrible TTT.

Hugs-

Julie

Edited September 8, 2007 by flop
Long quote removed