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Updates- 2009
Kelli's Page
December 2009
We had a fabulous Christmas though Kelli is still suffering with stomach issues. In fact, they
have gotten worse and it is becoming increasingly difficult for her to keep her night feed down.
because I plan to figure it out!
September 22, 2009
I took Kelli in to visit the doctor on the 14th because she had been lethargic all weekend and
had developed a nasty cough. After completing PFTs and having very low scores, they
decided to hospitalize her. Cultures showed that she was infected with both Influenza A and
H1N1. Luckily, they started her on treatments for both types of flues that day and by the time
the cultures came back positive she was pretty much done with the treatments. She came
home from the hospital on the 17th. However, the cultures they took on Monday the 21st have
not come back yet and we do not know yet if she is virus free. Therefore, she is spending the
week at home doing homework. Besides that, her classroom is full of coughing kids, so I
would just rather she stay home anyway. She is feeling great, looks good, and has regained
her appetite! So things are looking up. Thanks for all the prayers and phone calls! They
ALWAYS help!
September 10, 2009
As of today's date, Kelligar has gained 11 pounds! She has gained about 25% of her body
weight in a about 3 month's time. Her face is looking rather full, her arms and legs are a
"little" bit meatier and she has a little belly roll. Now we're just waiting for a growth spurt!
August 31, 2009 - Kelli's lung diseased identified
Someone asked me today about whether or not Kelligar's lung disease had ever been
identified. After looking through the Medical Updates sections I realized nothing was ever said
about it. It is named, ABCA3, which is a fatal surfactant deficiency. Surfactant is a mixture of
lipids and certain proteins in the lungs that basically keeps them from sticking together when
you exhale. It is my understanding and humbly and hopefully correctly described as the ABC
family which is a large family of proteins that occur across membranes. The ABCA subfamily
aids in the movement of phospholipids and cholesterol within and between the cells. The A3
transporter member directs the synthesis of a specific amino-acid protein. Although they don’t
quite know what the function of ABCA3 is, they believe that is involved in the movement of one
or more parts of surfactant in or out of the membranes. It is thought that this may eventually
be the most common genetic cause of lung disease in newborns.
Similar to other surfactant diseases and diseases like cystic fibrosis, each parent carries one
recessive gene. These coupled genes cause the disease in the infant. The deal here is, that
if both of us carried the recessive gene then all of our children would have been born with this
disease. However, only one out of three kids was born with this lung disease. The kicker
here is, Kevin does not carry the gene, only I do. Then the next logical question is why then did
Kelli get this lung disease? Well, for some unknown reason there was what is called a
"functioning gene mutation" on that particular gene, which in essence created a secondary
recessive gene. Therefore Kelli very unluckily received the two recessive genes necessary for
her to be born with this disease.
Since the cause has been identified, we have had the opportunity to have some testing done.
There was a 50/50 chance that I would pass that recessive gene onto my kids and
unfortunately, all received that gene and have potential to pass it on to their children.
August 2, 2009
THE BIG DAY! Kelli's party seemed to be a smash hit. "Into" her dog these days, she had a
dog themed party and had a cake shaped like a dog bone with "Happy Birthday Dog Breath"
on it. She loved it. I think 45 people showed up and we at, drank, and made merry. Turning
double digits was a monumental event for her. And us too!
August 1, 2009
We combined Kelligar's St. Louis check up with a family road trip. The trip was very eventful as
we decided to take family road trip and sight see along the way back. It started out eventful -
as is typical for vacations! - as we realized when we stopped for dinner in New Mexico that we
had left Kelli's meds in Phoenix. We mis-judged the length of time it would take to drive
straight through to Tulsa and ended up driving throughout the night. While on the road, I
contacted our transplant coordinator who happened to be on call, to tell her that we somehow
left the meds at home. I told her about the hospital in Tulsa and thought perhaps we could get
Kelli's medication filled there as a lot of places do not carry immuno-suppressents. Although
the hospital couldn't fill them, she did find a pharmacy that could. So totally exhausted and
grouchy, we made our way to the pharmacy, managing to miss only the nighttime dose. The
fun only increased after we told Kelli she'd have to learn to take pills because they could not
mix it into liquid form like at home. So sitting in the back of the minivan in humid Tulsa
weather in a Wal-Mart parking lot, armed with a water bottle - kicking and screaming - she
managed to swallow the pills! Kevin and the boys escaped into the confines of the Wal-Mart
store and managed to miss all the fun!
We made into St. Louis later in the day and relaxed for the rest of the night. The next day we
spent entirely at the Magic House. For all of you who don't know what it is, well, it's a one-of-a-
kind play house that even adults can have fun in. Kelli proceeded onto testing for the next
three days. Everything was pretty much uneventful with the exception of her Tac level being
almost triple what it should be. Very puzzling. With tests complete, we trudged onto
Colorado. We took the crooked and somewhat treacherous (in my opinion) road to the
Continental Divide and then spent a delightful day in out of rain showers at Vail, Aspen, and
ended the day at the wonderful Hotel Colorado in Glenwood Springs. Somewhere it dawned
on me why her Tac level was off. I checked the bottle and realized that the dose had been
mixed wrong, so we had basically double dosed three times in a row! Next day we shoved off
to Bryce Canyon in Utah. We visited every glorious site until Aidan exclaimed he was bored to
death. Again we drove through a good share of the night to the north rim at the Grand Canyon
Lodge. It was a very slow trip because the landscape (and road) was dotted with a gazillion
deer. Stayed in a great cabin which my city kids realized came equipped without a television!
After a fantastic breakfast and some walk abouts, we started our trip home. Made it home
earlier than anticipated which gave me just enough time to do some last minute shopping for
Kelligar's 10th Birthday Swim Party which was taking place the next day. GREAT planning on
my part!
July 14, 2009
The new year brought with it the clear indication that Kelligar was suffering from some
stomach issues. When we transferred Kelli's care from St. Louis Children's Hospital to the
Heart & Lung Institute here in Phoenix, she was already having difficulty gaining weight. At the
end of last year, we finally decided that enough was enough and the kid underwent a battery of
tests to determine what exactly was wrong. The following is our understanding of what
happened and the reason corrective surgery was absolutely necessary. Kelli was in the
hospital for 6 days but is home and doing wonderfully. As of today's date, she has gained 5
pounds!
At the time Kelli had her transplant they also did a surgery called a Nissen Fundoplication and
at the same time they put in her "button". The picture below shows how they do a nissen
fundo. Basically, they take the top portion of the stomach and wrap it around the bottom of the
esophagus just above the esophageal sphincter and stitch it in place (as seen in the picture
on the right). This basically prevents stomach acids from coming up the esophagus. Usually
with kids this fundo stretches with the child as they grow and generally doesn't cause any
issues. However, Kelli's nissen has slipped and is basically tightened around the top of the
stomach, in essence strangling her stomach, which makes it harder for food to get into her
stomach.
In addition to this, gastroparesis has occurred caused by the following. It appears that the
vagus nerve was damaged, probably at the time of transplant, which affects food digestion,
and it appears the pylorus (the lower tube of the stomach going into the small intestine) is
constricted. This means that her stomach really doesn't empty from meal to meal so she
really is full always. The following describes how the stomach works and what all the
terminology means.
Gastroparesis (gastric = stomach; paresis = paralysis) literally means stomach paralysis. It is
a condition in which the stomach muscle becomes slow and weakened. Following a meal, it
takes too long for the stomach to empty its contents into the small intestine.
How your stomach works. Most people don't know that the stomach lies high in the left upper
abdomen protected by the lower rib cage. Empty, the volume of the stomach "pouch" is less
than one-half cup. As you eat, the stomach's muscular wall can relax and expand to hold
about three pints of sustenance. The stomach's job is to liquify solid food preparing it for
digestion and absorption in the small intestine. This is done by mixing the food with powerful
digestive juices for several hours. To hold the food within the stomach there are two valves. At
the top of the stomach is the lower esophageal sphincter (LES) which prevents backsplash of
stomach contents upward into the esophagus. At the bottom of the stomach is the pylorus
which controls the "drain" of the stomach.
Once these two valves are closed, muscular contractions called peristaltic waves ripple
through the stomach squeezing gently in the upper part (fundus), more powerfully lower down
(antrum). These contractions are controlled by a stomach pacemaker, much like the heart,
and travel through the fibers of the vagus nerve. When the pacemaker fires, this muscular
churning motion mixes the food particles with powerful hydrochloric acid and the enzyme,
pepsin. Produced by the stomach, these strong chemicals convert the food to about the
consistency of cream of potato soup.
Eventually the pylorus relaxes slightly, opening the stomach's drain. The stomach muscle
contracts and the now liquefied food is pumped a little bit at a time through the valve and into
the small intestine where the digestive process occurs.
When all is working, the fullness we feel after eating a big meal gradually fades as the
stomach empties its contents into the small intestine. After a few hours, the stomach is
completely empty and ready for the next meal.
What if the stomach pacemaker slows or the pump fails? Then the stomach drains much
more slowly and does not empty completely between meals. With the next meal, you would
feel bloated and perhaps nauseated. There would be no room for more food and vomiting of
undigested food might occur. Each meal would be an ordeal. You might be afraid to eat and
begin to lose some weight. This is what patients with gastroparesis have to put up with each
day. This is what happened to Kelli.
We had a fabulous Christmas though Kelli is still suffering with stomach issues. In fact, they
have gotten worse and it is becoming increasingly difficult for her to keep her night feed down.
because I plan to figure it out!
September 22, 2009
I took Kelli in to visit the doctor on the 14th because she had been lethargic all weekend and
had developed a nasty cough. After completing PFTs and having very low scores, they
decided to hospitalize her. Cultures showed that she was infected with both Influenza A and
H1N1. Luckily, they started her on treatments for both types of flues that day and by the time
the cultures came back positive she was pretty much done with the treatments. She came
home from the hospital on the 17th. However, the cultures they took on Monday the 21st have
not come back yet and we do not know yet if she is virus free. Therefore, she is spending the
week at home doing homework. Besides that, her classroom is full of coughing kids, so I
would just rather she stay home anyway. She is feeling great, looks good, and has regained
her appetite! So things are looking up. Thanks for all the prayers and phone calls! They
ALWAYS help!
September 10, 2009
As of today's date, Kelligar has gained 11 pounds! She has gained about 25% of her body
weight in a about 3 month's time. Her face is looking rather full, her arms and legs are a
"little" bit meatier and she has a little belly roll. Now we're just waiting for a growth spurt!
August 31, 2009 - Kelli's lung diseased identified
Someone asked me today about whether or not Kelligar's lung disease had ever been
identified. After looking through the Medical Updates sections I realized nothing was ever said
about it. It is named, ABCA3, which is a fatal surfactant deficiency. Surfactant is a mixture of
lipids and certain proteins in the lungs that basically keeps them from sticking together when
you exhale. It is my understanding and humbly and hopefully correctly described as the ABC
family which is a large family of proteins that occur across membranes. The ABCA subfamily
aids in the movement of phospholipids and cholesterol within and between the cells. The A3
transporter member directs the synthesis of a specific amino-acid protein. Although they don’t
quite know what the function of ABCA3 is, they believe that is involved in the movement of one
or more parts of surfactant in or out of the membranes. It is thought that this may eventually
be the most common genetic cause of lung disease in newborns.
Similar to other surfactant diseases and diseases like cystic fibrosis, each parent carries one
recessive gene. These coupled genes cause the disease in the infant. The deal here is, that
if both of us carried the recessive gene then all of our children would have been born with this
disease. However, only one out of three kids was born with this lung disease. The kicker
here is, Kevin does not carry the gene, only I do. Then the next logical question is why then did
Kelli get this lung disease? Well, for some unknown reason there was what is called a
"functioning gene mutation" on that particular gene, which in essence created a secondary
recessive gene. Therefore Kelli very unluckily received the two recessive genes necessary for
her to be born with this disease.
Since the cause has been identified, we have had the opportunity to have some testing done.
There was a 50/50 chance that I would pass that recessive gene onto my kids and
unfortunately, all received that gene and have potential to pass it on to their children.
August 2, 2009
THE BIG DAY! Kelli's party seemed to be a smash hit. "Into" her dog these days, she had a
dog themed party and had a cake shaped like a dog bone with "Happy Birthday Dog Breath"
on it. She loved it. I think 45 people showed up and we at, drank, and made merry. Turning
double digits was a monumental event for her. And us too!
August 1, 2009
We combined Kelligar's St. Louis check up with a family road trip. The trip was very eventful as
we decided to take family road trip and sight see along the way back. It started out eventful -
as is typical for vacations! - as we realized when we stopped for dinner in New Mexico that we
had left Kelli's meds in Phoenix. We mis-judged the length of time it would take to drive
straight through to Tulsa and ended up driving throughout the night. While on the road, I
contacted our transplant coordinator who happened to be on call, to tell her that we somehow
left the meds at home. I told her about the hospital in Tulsa and thought perhaps we could get
Kelli's medication filled there as a lot of places do not carry immuno-suppressents. Although
the hospital couldn't fill them, she did find a pharmacy that could. So totally exhausted and
grouchy, we made our way to the pharmacy, managing to miss only the nighttime dose. The
fun only increased after we told Kelli she'd have to learn to take pills because they could not
mix it into liquid form like at home. So sitting in the back of the minivan in humid Tulsa
weather in a Wal-Mart parking lot, armed with a water bottle - kicking and screaming - she
managed to swallow the pills! Kevin and the boys escaped into the confines of the Wal-Mart
store and managed to miss all the fun!
We made into St. Louis later in the day and relaxed for the rest of the night. The next day we
spent entirely at the Magic House. For all of you who don't know what it is, well, it's a one-of-a-
kind play house that even adults can have fun in. Kelli proceeded onto testing for the next
three days. Everything was pretty much uneventful with the exception of her Tac level being
almost triple what it should be. Very puzzling. With tests complete, we trudged onto
Colorado. We took the crooked and somewhat treacherous (in my opinion) road to the
Continental Divide and then spent a delightful day in out of rain showers at Vail, Aspen, and
ended the day at the wonderful Hotel Colorado in Glenwood Springs. Somewhere it dawned
on me why her Tac level was off. I checked the bottle and realized that the dose had been
mixed wrong, so we had basically double dosed three times in a row! Next day we shoved off
to Bryce Canyon in Utah. We visited every glorious site until Aidan exclaimed he was bored to
death. Again we drove through a good share of the night to the north rim at the Grand Canyon
Lodge. It was a very slow trip because the landscape (and road) was dotted with a gazillion
deer. Stayed in a great cabin which my city kids realized came equipped without a television!
After a fantastic breakfast and some walk abouts, we started our trip home. Made it home
earlier than anticipated which gave me just enough time to do some last minute shopping for
Kelligar's 10th Birthday Swim Party which was taking place the next day. GREAT planning on
my part!
July 14, 2009
The new year brought with it the clear indication that Kelligar was suffering from some
stomach issues. When we transferred Kelli's care from St. Louis Children's Hospital to the
Heart & Lung Institute here in Phoenix, she was already having difficulty gaining weight. At the
end of last year, we finally decided that enough was enough and the kid underwent a battery of
tests to determine what exactly was wrong. The following is our understanding of what
happened and the reason corrective surgery was absolutely necessary. Kelli was in the
hospital for 6 days but is home and doing wonderfully. As of today's date, she has gained 5
pounds!
At the time Kelli had her transplant they also did a surgery called a Nissen Fundoplication and
at the same time they put in her "button". The picture below shows how they do a nissen
fundo. Basically, they take the top portion of the stomach and wrap it around the bottom of the
esophagus just above the esophageal sphincter and stitch it in place (as seen in the picture
on the right). This basically prevents stomach acids from coming up the esophagus. Usually
with kids this fundo stretches with the child as they grow and generally doesn't cause any
issues. However, Kelli's nissen has slipped and is basically tightened around the top of the
stomach, in essence strangling her stomach, which makes it harder for food to get into her
stomach.
In addition to this, gastroparesis has occurred caused by the following. It appears that the
vagus nerve was damaged, probably at the time of transplant, which affects food digestion,
and it appears the pylorus (the lower tube of the stomach going into the small intestine) is
constricted. This means that her stomach really doesn't empty from meal to meal so she
really is full always. The following describes how the stomach works and what all the
terminology means.
Gastroparesis (gastric = stomach; paresis = paralysis) literally means stomach paralysis. It is
a condition in which the stomach muscle becomes slow and weakened. Following a meal, it
takes too long for the stomach to empty its contents into the small intestine.
How your stomach works. Most people don't know that the stomach lies high in the left upper
abdomen protected by the lower rib cage. Empty, the volume of the stomach "pouch" is less
than one-half cup. As you eat, the stomach's muscular wall can relax and expand to hold
about three pints of sustenance. The stomach's job is to liquify solid food preparing it for
digestion and absorption in the small intestine. This is done by mixing the food with powerful
digestive juices for several hours. To hold the food within the stomach there are two valves. At
the top of the stomach is the lower esophageal sphincter (LES) which prevents backsplash of
stomach contents upward into the esophagus. At the bottom of the stomach is the pylorus
which controls the "drain" of the stomach.
Once these two valves are closed, muscular contractions called peristaltic waves ripple
through the stomach squeezing gently in the upper part (fundus), more powerfully lower down
(antrum). These contractions are controlled by a stomach pacemaker, much like the heart,
and travel through the fibers of the vagus nerve. When the pacemaker fires, this muscular
churning motion mixes the food particles with powerful hydrochloric acid and the enzyme,
pepsin. Produced by the stomach, these strong chemicals convert the food to about the
consistency of cream of potato soup.
Eventually the pylorus relaxes slightly, opening the stomach's drain. The stomach muscle
contracts and the now liquefied food is pumped a little bit at a time through the valve and into
the small intestine where the digestive process occurs.
When all is working, the fullness we feel after eating a big meal gradually fades as the
stomach empties its contents into the small intestine. After a few hours, the stomach is
completely empty and ready for the next meal.
What if the stomach pacemaker slows or the pump fails? Then the stomach drains much
more slowly and does not empty completely between meals. With the next meal, you would
feel bloated and perhaps nauseated. There would be no room for more food and vomiting of
undigested food might occur. Each meal would be an ordeal. You might be afraid to eat and
begin to lose some weight. This is what patients with gastroparesis have to put up with each
day. This is what happened to Kelli.
