advise please - 13yr old just over 7th pneumonia

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HuntersmomTBM

New member
Hello All,
I am in need of advise. My son, Hunter is 13 his current full diagnosed is Tracheobronchomalacia (TBM) and Innominate Artery Compression (IAC), Reflux and Reactive Airway Disease. TBM=softened and misshaped airway causing collasaping. IAC=an artery from his heart compresses his trachea.
He has had 1RSV, 7Pneumonias, 6Bronchitis' and 15Croups -plus coutless other URIs that weren't given a name.
He was first tested for CF in 2005, he was borderline at 40. His ex-pulmonary dr did nothing. When he closed his local office he sent all the paperwork to his dr. He was re-tested -sweat and blood test -both were negative.
Now we are awaiting results for the genetic test. They are looking for Poly-T Variant. He is currently on Flovent daily, stool softner daily, acid reflux meds daily, Atrovent PRN, Xopenex to follow PRN and steriod pills at sign of first illness. Of course I am worried. I have most of his medical records and PFTs at the house.
Is there anything I should look for? I've been reading about low results FEV1 -his last FEV1 was 92. His problem is FIF Max (L/sec) base is 68. His FEF 75% is 63. His PFT's show blunting.
Mother with COPD and mother in law with lung cancer. Should he stay away from them?
Colleen
mother to Dominick (15), Hunter (13 TBM & IAC) and Jennifer (12)
<strong>-------------------------</strong> Colleen mom to Dominick 15 ,Hunter 13 (Tracheobronchomalacia & Innominate Artery Compression) and Jennifer 12
 
H

HuntersmomTBM

New member
Hello All,
I am in need of advise. My son, Hunter is 13 his current full diagnosed is Tracheobronchomalacia (TBM) and Innominate Artery Compression (IAC), Reflux and Reactive Airway Disease. TBM=softened and misshaped airway causing collasaping. IAC=an artery from his heart compresses his trachea.
He has had 1RSV, 7Pneumonias, 6Bronchitis' and 15Croups -plus coutless other URIs that weren't given a name.
He was first tested for CF in 2005, he was borderline at 40. His ex-pulmonary dr did nothing. When he closed his local office he sent all the paperwork to his dr. He was re-tested -sweat and blood test -both were negative.
Now we are awaiting results for the genetic test. They are looking for Poly-T Variant. He is currently on Flovent daily, stool softner daily, acid reflux meds daily, Atrovent PRN, Xopenex to follow PRN and steriod pills at sign of first illness. Of course I am worried. I have most of his medical records and PFTs at the house.
Is there anything I should look for? I've been reading about low results FEV1 -his last FEV1 was 92. His problem is FIF Max (L/sec) base is 68. His FEF 75% is 63. His PFT's show blunting.
Mother with COPD and mother in law with lung cancer. Should he stay away from them?
Colleen
mother to Dominick (15), Hunter (13 TBM & IAC) and Jennifer (12)
<strong>-------------------------</strong> Colleen mom to Dominick 15 ,Hunter 13 (Tracheobronchomalacia & Innominate Artery Compression) and Jennifer 12
 
H

HuntersmomTBM

New member
Hunter has also been to the ER twice for constipation. He was dx with Failure to Thrive as a baby. He's been hospitalized 4 -times for a week each time. To the ER at least 35 times. His PFT posted above are when he was well.
 
H

HuntersmomTBM

New member
Hunter has also been to the ER twice for constipation. He was dx with Failure to Thrive as a baby. He's been hospitalized 4 -times for a week each time. To the ER at least 35 times. His PFT posted above are when he was well.
 
J

Julie7

New member
Colleen,
I'm sorry you have these health issues with Hunter, I am sure you are stressed about all this. I'm sure others will also recommend that you are being seen by a certified CF center and possibly you should seek out one of the larger centers that would offer the best care. When you say you are awaiting genetics, do you know if they are screening for as much as possible, like the Ambry amplified panel many refer to on these boards? How are his physical stats now, given he was FFT as a baby - has he grown normally? Does he complain of any abdominal pain or diarrhea? CF affects the GI system as well as the lungs. Regardless, are you satisfied with his pulminologist? Perhaps you might need another one of those too. I am in the waiting game too on the genetics. My son is 8, he has pancreatic insufficiency, with little lung problems outside of lots of sinusitis/nasal infx problems. This is week 3 for us, I've heard to expect results in 6 weeks, another 3 to go. There are a ton of great people on these boards that are quite knowledgeable so I'm sure you will get additional advise from parents with similar stories.
Wish you the best for Hunter.
 
J

Julie7

New member
Colleen,
I'm sorry you have these health issues with Hunter, I am sure you are stressed about all this. I'm sure others will also recommend that you are being seen by a certified CF center and possibly you should seek out one of the larger centers that would offer the best care. When you say you are awaiting genetics, do you know if they are screening for as much as possible, like the Ambry amplified panel many refer to on these boards? How are his physical stats now, given he was FFT as a baby - has he grown normally? Does he complain of any abdominal pain or diarrhea? CF affects the GI system as well as the lungs. Regardless, are you satisfied with his pulminologist? Perhaps you might need another one of those too. I am in the waiting game too on the genetics. My son is 8, he has pancreatic insufficiency, with little lung problems outside of lots of sinusitis/nasal infx problems. This is week 3 for us, I've heard to expect results in 6 weeks, another 3 to go. There are a ton of great people on these boards that are quite knowledgeable so I'm sure you will get additional advise from parents with similar stories.
Wish you the best for Hunter.
 
H

HuntersmomTBM

New member
Julie7,
Thank you for your response. Hunter is not growing normally. He is small for age. He has a brother that is 22months older, D is 5'11" 180lbs Hunter is 5' and just hit 110lbs. He is about to be 14. We have never seen the big growth spurts like we do with other boys his age. He does grow but it's more slow and steady. He is usually in the 20-40%., but he dipped below the growth chart when he was a toddler for a short time. His brother D was 95% or off the charts. His sister, J is 16 months she is taller but her weight is 102lbs.
 
H

HuntersmomTBM

New member
Julie7,
Thank you for your response. Hunter is not growing normally. He is small for age. He has a brother that is 22months older, D is 5'11" 180lbs Hunter is 5' and just hit 110lbs. He is about to be 14. We have never seen the big growth spurts like we do with other boys his age. He does grow but it's more slow and steady. He is usually in the 20-40%., but he dipped below the growth chart when he was a toddler for a short time. His brother D was 95% or off the charts. His sister, J is 16 months she is taller but her weight is 102lbs.
 
P

Printer

Active member
He NEEDS to be seen at an APPROVED CF CENTER by an CF SPECIALIST. He NEEDS to have a FULL CF SEQUENCING done.

I'm not joking when I say his life is in the balance.

Bill
 
P

Printer

Active member
He NEEDS to be seen at an APPROVED CF CENTER by an CF SPECIALIST. He NEEDS to have a FULL CF SEQUENCING done.

I'm not joking when I say his life is in the balance.

Bill
 
H

HuntersmomTBM

New member
Bill,

I believe you. We have almost lost Hunter 4 times. I feel like I am yelling at the top of my lungs something is wrong and no one is listening. Hunter did get the genetic CF test done, we are on week 5 of waiting for results. When you say Approved CF Center -how do you find out which hospital is? We live in Southern Maryland and go to Children's National Medical Center in DC. After talking to another mom, whose child has Tracheomalacia and Innominate Artery Compression and CF -she highly recommended we go to Children's Hospital of Philadelphia. My husband and I are originally from Philly. So that idea has occurred to us. We are also looking at Boston's Children Hospital as they are the leader in the country for a surgery to repair the Innominate Artery Compression. But after talking further to "my new friend" I requested an appointment on line at CHOP. Please, do not hesitate to give me advise I need it. Do you or anyone else have any other advise for me??
~Colleen
 
H

HuntersmomTBM

New member
Bill,

I believe you. We have almost lost Hunter 4 times. I feel like I am yelling at the top of my lungs something is wrong and no one is listening. Hunter did get the genetic CF test done, we are on week 5 of waiting for results. When you say Approved CF Center -how do you find out which hospital is? We live in Southern Maryland and go to Children's National Medical Center in DC. After talking to another mom, whose child has Tracheomalacia and Innominate Artery Compression and CF -she highly recommended we go to Children's Hospital of Philadelphia. My husband and I are originally from Philly. So that idea has occurred to us. We are also looking at Boston's Children Hospital as they are the leader in the country for a surgery to repair the Innominate Artery Compression. But after talking further to "my new friend" I requested an appointment on line at CHOP. Please, do not hesitate to give me advise I need it. Do you or anyone else have any other advise for me??
~Colleen
 
P

Printer

Active member
Colleen:

There are so many levels of genetic testing. When I first had Genetic Testing, there was 250 known mutations, today there are more than 1800. He needs to be tested for the FULL CF SEQUENCING. I go to the Adult Clinic at Children's Hospital of Boston. All of the Doctors there are great. I have heard good things about Childrens in Philly also. You would do well with either.

Doctor Hank Dorkin (at Children's in Boston) was recently named the best Peadiactric Puminary Doctor in Massachusetts (he is a CF Specialist and a great guy).

Only extensive testing will tell you if he has CF. Boston will likely want a referral from your Doctor, the Referral FAX number is 617-730-0373 and the Scheduling Number is 617-355-1900.

Boston is a great city to visit.

Bill
 
P

Printer

Active member
Colleen:

There are so many levels of genetic testing. When I first had Genetic Testing, there was 250 known mutations, today there are more than 1800. He needs to be tested for the FULL CF SEQUENCING. I go to the Adult Clinic at Children's Hospital of Boston. All of the Doctors there are great. I have heard good things about Childrens in Philly also. You would do well with either.

Doctor Hank Dorkin (at Children's in Boston) was recently named the best Peadiactric Puminary Doctor in Massachusetts (he is a CF Specialist and a great guy).

Only extensive testing will tell you if he has CF. Boston will likely want a referral from your Doctor, the Referral FAX number is 617-730-0373 and the Scheduling Number is 617-355-1900.

Boston is a great city to visit.

Bill
 
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