Bad week-updates for Asenath and Zipporrah

[Guest guest]

This has been another tiring, long week filled with more difficult issues to swallow. I will try to make this brief and ask a few questions as well. I appreciate those who take the time to read and possibly respond if possible. Asenath Asenath thankfully does not appear to have an ulcer or gastro-enteralitis (spelling off) and no reflux has been seen as a possibility for the vomiting She still has a few more tests to check for gastro-issues later in July. The worrisome issue for her was during the Pulmonology appointment. The doc said he thought is highly unlikely that her problems are asthma related; one reason being her high steroid use for the past year. Over the past few months Asenath has been coughing more and more anytime she walks around, her pulse-ox is lower, and she gets breathless. The doc suggested it could be a weakness in her muscles and part of the Mito progression. I really didn't like hearing this and honestly it has frightened me quite a bit. It seems there may be nothing that can help it at this point but the ENT visit we scheduled for the end of July was exactly what the doc suggested us to do so I am glad we did schedule it already. He wants her trachea checked out and other breathing areas to see if they can identify the problem. I will update you on this as we do the testing/doc visits. What is your experience with these issues? Have you seen more breathing problems with progression of Mito? What has helped your kids? Have you seen lots of coughing with movement in your kids? Zipporrah The dumping syndrome is quite significant we found out and the doc wants to discuss the issue further with Dr. Whiteman to see what all this can affect in Mito (she is an excellent doctor but new to Mayo and probably to Mito somewhat). She has been very thorough with both girls and has suggested we try adding Benefiber to her formula to see if that helps Is that a helpful method for dumping? What else have you done to control dumping? What all does dumping affect in Mito kids? The doc suggested trying the fiber before going to 24 hour feeds as feeding continually can be so restrictive at this age to Zipporrah. I am also worried that if the dumping is this bad, how the meds are going to be absorbed. Another issue is the tracheal ring. We have not discussed it with the ENT yet but the GI doc and the Pulmo. doc both saw the photos and said she does have the subclavian valve (I think) leaning against the trachea causing some compression. They both felt it wasn't bad enough for surgery, but wondered how it affects her during asthma, viruses, or during sleep/crying. I hope to get more specifics later on August 4th Finally, the pulmo. doc, even though seeing Aseanth, took a look at Zipporrah as she was making funny noises and asked about her issues. After hearing about all her breathing issues he mentioned he thought she might have a partial paralysis of her vocal chords. He wants to talk to the ENT about her (as well as Asenath) and do further studies, probably while awake to see what all is happening with her airways. Anyone have experience with this issue? Can anything help? I am still trying to process everything and overwhelmed by it all. It seems as if the strokes in both girls are affecting breathing/airways more than I even thought. Please continue to pray for our girls. I am soooooo glad to be home. Darla: mommy to: Asenath (4) Mito, CNS Vasculitis, strokes, migraines, seizures, G-tube, hypotonicity, disautonomy,SID, dev. delays, breathing/tracheal issues? Zipporrah (7 mon) Mito, strokes, SID, G-tube, asthma, trachea issues, disautonomy, hypo & hypertonicity, migraines and the gangGet more from the Web. FREE MSN Explorer download : http://explorer.msn.com

[Guest guest]

Hi Darla-

Prayers are coming your way. I also just e-mailed a friend of mine from the Syndrome listserve I used to be on (2 years ago before we knew about mito, Maddie had suspected WS.) Anyhow- her daughter was born with paralyzed vocal chords, and she has done lots of research in many different areas regarding . I'm hoping to put the two of you in contact with each other, if you'd like. Here is 's website http://home.san.rr.com/krowe/

It looks as though has had a life changing surgery, allowing her trach (which she's had since birth) to be removed and dramatically changing her quality of life. I'm not sure whether Porrahs situation is the same, but Kathy is the only person I know of who has dealt with paralyzed vocal chords. She and her daughter both have strong, incredible spirits, and she (like you) amazes me. Be strong in your faith, and let me know if you'd like to get in contact with her.

Holly Rubio

Bad week-updates for Asenath and Zipporrah

This has been another tiring, long week filled with more difficult issues to swallow. I will try to make this brief and ask a few questions as well. I appreciate those who take the time to read and possibly respond if possible.

Asenath

Asenath thankfully does not appear to have an ulcer or gastro-enteralitis (spelling off) and no reflux has been seen as a possibility for the vomiting She still has a few more tests to check for gastro-issues later in July. The worrisome issue for her was during the Pulmonology appointment. The doc said he thought is highly unlikely that her problems are asthma related; one reason being her high steroid use for the past year. Over the past few months Asenath has been coughing more and more anytime she walks around, her pulse-ox is lower, and she gets breathless. The doc suggested it could be a weakness in her muscles and part of the Mito progression. I really didn't like hearing this and honestly it has frightened me quite a bit. It seems there may be nothing that can help it at this point but the ENT visit we scheduled for the end of July was exactly what the doc suggested us to do so I am glad we did schedule it already. He wants her trachea checked out and other breathing areas to see if they can identify the problem. I will update you on this as we do the testing/doc visits. What is your experience with these issues? Have you seen more breathing problems with progression of Mito? What has helped your kids? Have you seen lots of coughing with movement in your kids?

Zipporrah

The dumping syndrome is quite significant we found out and the doc wants to discuss the issue further with Dr. Whiteman to see what all this can affect in Mito (she is an excellent doctor but new to Mayo and probably to Mito somewhat). She has been very thorough with both girls and has suggested we try adding Benefiber to her formula to see if that helps Is that a helpful method for dumping? What else have you done to control dumping? What all does dumping affect in Mito kids? The doc suggested trying the fiber before going to 24 hour feeds as feeding continually can be so restrictive at this age to Zipporrah. I am also worried that if the dumping is this bad, how the meds are going to be absorbed.

Another issue is the tracheal ring. We have not discussed it with the ENT yet but the GI doc and the Pulmo. doc both saw the photos and said she does have the subclavian valve (I think) leaning against the trachea causing some compression. They both felt it wasn't bad enough for surgery, but wondered how it affects her during asthma, viruses, or during sleep/crying. I hope to get more specifics later on August 4th

Finally, the pulmo. doc, even though seeing Aseanth, took a look at Zipporrah as she was making funny noises and asked about her issues. After hearing about all her breathing issues he mentioned he thought she might have a partial paralysis of her vocal chords. He wants to talk to the ENT about her (as well as Asenath) and do further studies, probably while awake to see what all is happening with her airways. Anyone have experience with this issue? Can anything help?

I am still trying to process everything and overwhelmed by it all. It seems as if the strokes in both girls are affecting breathing/airways more than I even thought. Please continue to pray for our girls. I am soooooo glad to be home.

Darla: mommy to:

Asenath (4) Mito, CNS Vasculitis, strokes, migraines, seizures, G-tube, hypotonicity, disautonomy,SID, dev. delays, breathing/tracheal issues?

Zipporrah (7 mon) Mito, strokes, SID, G-tube, asthma, trachea issues, disautonomy, hypo & hypertonicity, migraines

and the gangPlease contact mito-owner with any problems or questions.

Get more from the Web. FREE MSN Explorer download : http://explorer.msn.com

[Guest guest]

Darla,

I am so sorry that you and the kids have had such a hard time. I

have no advice except that I would think the Benefiber would help to

bulk up the stools and intestinal contents making the dumping issue

less severe?!? I would definately try this. It is very hard to think

about this horrible beast of mito progressing. We try not to think

about it. We get into our daily groove and then there it is again.

My thoughts and prayers are with you as always,

Dawn

[Guest guest]

Darla : Our Prayers are with you . I am so sorry t hear about this I have no advice , but My family is praying for you Asenath and Zipporrah. God Bless and lol and LOL Jenniethefourofus03 wrote:

Darla, I am so sorry that you and the kids have had such a hard time. I have no advice except that I would think the Benefiber would help to bulk up the stools and intestinal contents making the dumping issue less severe?!? I would definately try this. It is very hard to think about this horrible beast of mito progressing. We try not to think about it. We get into our daily groove and then there it is again. My thoughts and prayers are with you as always, DawnPlease contact mito-owner with any problems or questions.

[Guest guest]

Sorry to hear about all of the breathing problems from the strokes.

Our thoughts and prayers are with you.

Geri-Anne and Wyatt, Complex I-

-- In Mito , " Darla Klein "

wrote:

> This has been another tiring, long week filled with more

difficult issues to swallow. I will try to make this brief and ask

a few questions as well. I appreciate those who take the time to

read and possibly respond if possible.

> Asenath

> Asenath thankfully does not appear to have an ulcer or

gastro-enteralitis (spelling off) and no reflux has been seen as a

possibility for the vomiting. She still has a few more tests to

check for gastro-issues later in July. The worrisome issue for her

was during the Pulmonology appointment. The doc said he thought is

highly unlikely that her problems are asthma related; one reason

being her high steroid use for the past year Over the past few

months Asenath has been coughing more and more anytime she walks

around, her pulse-ox is lower, and she gets breathless. The doc

suggested it could be a weakness in her muscles and part of the Mito

progression. I really didn't like hearing this and honestly it has

frightened me quite a bit. It seems there may be nothing that can

help it at this point but the ENT visit we scheduled for the end of

July was exactly what the doc suggested us to do so I am glad we did

schedule it already He wants her trachea checked out and other

breathing areas to see if they can identify the problem. I will

update you on this as we do the testing/doc visits. What is your

experience with these issues? Have you seen more breathing problems

with progression of Mito? What has helped your kids? Have you seen

lots of coughing with movement in your kids?

> Zipporrah

> The dumping syndrome is quite significant we found out and

the doc wants to discuss the issue further with Dr. Whiteman to see

what all this can affect in Mito (she is an excellent doctor but new

to Mayo and probably to Mito somewhat). She has been very thorough

with both girls and has suggested we try adding Benefiber to her

formula to see if that helps. Is that a helpful method for

dumping? What else have you done to control dumping? What all does

dumping affect in Mito kids? The doc suggested trying the fiber

before going to 24 hour feeds as feeding continually can be so

restrictive at this age to Zipporrah. I am also worried that if the

dumping is this bad, how the meds are going to be absorbed.

> Another issue is the tracheal ring. We have not discussed it

with the ENT yet but the GI doc and the Pulmo. doc both saw the

photos and said she does have the subclavian valve (I think) leaning

against the trachea causing some compression. They both felt it

wasn't bad enough for surgery, but wondered how it affects her

during asthma, viruses, or during sleep/crying. I hope to get more

specifics later on August 4th.

> Finally, the pulmo. doc, even though seeing Aseanth, took a

look at Zipporrah as she was making funny noises and asked about her

issues. After hearing about all her breathing issues he mentioned

he thought she might have a partial paralysis of her vocal chords.

He wants to talk to the ENT about her (as well as Asenath) and do

further studies, probably while awake to see what all is

happening with her airways. Anyone have experience with this

issue? Can anything help?

> I am still trying to process everything and overwhelmed by it

all. It seems as if the strokes in both girls are affecting

breathing/airways more than I even thought. Please continue to pray

for our girls. I am soooooo glad to be home.

>

>

> Darla: mommy to:

> Asenath (4) Mito, CNS Vasculitis, strokes, migraines, seizures, G-

tube, hypotonicity, disautonomy,SID, dev. delays, breathing/tracheal

issues?

> Zipporrah (7 mon.) Mito, strokes, SID, G-tube, asthma, trachea

issues, disautonomy, hypo & hypertonicity, migraines

> and the gangGet more from the Web. FREE MSN Explorer download :

http://explorer.msn.com

[Guest guest]

The worrisome issue for her was during the Pulmonology appointment. The

doc said he thought is highly unlikely that her problems are asthma related;

Asenath has been coughing more and more anytime she walks around, her

pulse-ox is lower, and she gets breathless. The doc suggested it could be a

weakness in her muscles and part of the Mito progression. It seems there

may be nothing that can help it at this point but the ENT visit we scheduled for

the end of July was exactly what the doc suggested us to do so I am glad we

did schedule it already

Darla,

Have they scheduled a sleep study? I would ask for one now--before the ENT

appointment. How is her serum carbon dioxide? Bicarb? Exhaled gasses? If

she needs breathing support there are noninvasive vents available for

children. Joanne Kocourek knows tons about available options. Noninvasive

vents may be better tolerated in mito children.

[Guest guest]

Darla,

I am sorry your visit was not full of good news. I really don't have any good advice for you, sorry. Our only experience with any of the girls issues is with the breathing. Grace has cough varient asthma. The difference is that she does not typically wheeze, she coughs and gets very congested in her chest. We discovered that allergies were the trigger. Much to our relief allergy medications keep her asthma really controlled. We still have times that we must resort to steroids (inhaled), but 80% of the time she does well. Her pulse ox is always low 95-96, but I have not been able to discuss this with her asthma doc yet to see if this is anything to be concerned about or not. I am just telling you this to help give you some hope. We were so worried about Gracies issues, and it turned out to be very minor. Hopefully this can happen with your girls. Those little sweethearts have to much on there hands already. They deserve to catch a break. Best wishes, hope the girls feel better really soon.

[Guest guest]

What is your experience with these issues? Have you seen more breathing problems with progression of Mito? What has helped your kids? Have you seen lots of coughing with movement in your kids?

Darla, With Chelsea, she has a lot of coughing issues related to position changes. Particularly when sitting upright from a laying position. She is non mobile and produces a lot of secretions, and also has the dyspagia playing a big part. What happens, is her secretion pool at the lowest spot and then when she is moved, the secretions drain and cause coughing. SHe also seems to do quite a bit of coughing shortly after going to bed most nites. The thicker her secretions are, the more likely she is to gag and wretch with suctioning. Her issues are from asthma, as we recently found out, and also due to compromised lung function from low muscle tone. She does not breathe deeply to fully fill her lungs and as a result does not fully clear the lower regions of her lungs. Her rib cage is very shallow as a result.

Fortunatly, Chelsea's secretion managment has improved over time (unlike what we were told when her gtube was placed 4 yrs ago). At one point, her cough was completely ineffective and she sounded like a drowning victim (I actually had people ask me if she was). With chest pt and meds and suctioning (which forces her to cough), her cough and ability to manage her secretions has improved to the point where she can cough it up and, sometimes, spit it out.

What else have you done to control dumping? What all does dumping affect in Mito kids? The doc suggested trying the fiber before going to 24 hour feeds as feeding continually can be so restrictive at this age to Zipporrah. I am also worried that if the dumping is this bad, how the meds are going to be absorbed.

Someone correct me if this is bad info, but I believe I have read that dumping syndrome can be caused by sugar intolerence. All fromulas tend to be high in refined sugar, which can cause havoc with bood sugar levels. Chelsea never did well on all Pediasure diet, she would have a short energy burst after feeding, and then be lethargic until her next feeding. She seems to be doing much better with complex carbs and very little simple sugars.

I am sorry to hear that more issues are popping up with your kids. You must be very overwhelmed. I will continue to keep you all in my prayers, hope you all are enjoying summer=)

e, Chelsea's mom(nonspecific mito)