Our family is preparing for a lot of new beginnings. Putting an era behind us, something many families do. A move is a new start, right? Today we are leaving even more behind.
Today Angela had a swallow study. Let me give you a Reader's Digest version of the past year.
Angela has always had trouble swallowing. As an infant and toddler she had a tube because she couldn't swallow liquids without aspirating them into her lungs. Then around age 7..or maybe 10...she was diagnosed with cricopharngeal achalasia. Lets just say, it is a really bad swallowing disorder. At 14 or so it got much worse and we had to seek treatment in Boston. (we live in MN).
We had 19 years of weird medical problems, manic behavior, aggression, seizures, stroke, TIA's. All chalked up to "well this can be part of Down syndrome." We swallowed that pill and always moved forward. Angela was on a slew of antipsychotic medications.
Then in April 2015 she developed aspiration pneumonia. Then again in July...and September. We did a video swallow, stunned to find she had lost the ability to swallow ANYTHING safely. Absolutely everything went into her lungs, and solid foods stuck right in front of her airway. Her swallow was completely uncoordinated and dangerous for her. We were devastated, and telling her she couldn't eat anymore was beyond hard.
In October she had a tube placed. She entered the hospital her peppy, sassy self, and left on her very special formula depressed. She stopped smiling and started sleeping all the time. We called it depression until one day she wouldn't wake up. We found her on the floor of her room, face down in the carpet. I thought she was dead. She wasn't, she was in a coma.
And ambulance ride. Can't get an airway. Intubated. Weird IV line thingy jammed in her leg (what the hell was THAT? It looks weeks to heal!) 24 hours and she was awake and smiling. Nobody knew why, but I suspected it was some kind of reaction to that amazing formula we put her on.
Two weeks. Another coma. Lots of head scratching and even some finger pointing (Maybe we drugged her?) I refused to take her home until they figured it out. I told them to give her that formula, there was a problem, I knew it. A call to metabolic specialists lead to the first ammonia test, which was the super fast ticket to the Intensive Care Unit and a rescue drug that would clear the ammonia from her blood....at the risk of killing her kidneys.
A whole new life of protein restriction because her liver couldn't process it. Crazy formula and drugs to the tune of $30,000 per month. Potential rare disease diagnosis (OTC deficiency) while we waited for the necessary genetic testing. More ICU stays. More confusion. No longer responding to the rescue drug. One smart doctor who never stopped digging, on a whim ordered a liver scan.
There it was. Abernathy Type II malformation. The rarest of rare liver malformations. Blood not going where it needed to go so the liver couldn't do its job. A move to a different hospital who would know what to do. They sent us home while they researched a possible fix. Consulting doctors all over the country to come up with a plan.
April 8th. An experimental procedure they've never tried before. It worked!!!! She is a whole new person!! Manic moods gone. Aggression gone. Seizures gone. Happy. Always smiling. A totally new person. Maybe her swallow was better too? Ammonia affects muscle tone and control, maybe it was the cause of the swallowing problems? It was a long shot. We started letting her eat more. Drinking liquids. She was doing it without developing pneumonias. No sputtering. No choking.
And then today. A new swallow study. Truly stunned.
She passed.
100% oral eating as of today. She is whole again.
She's not done. She has one more surgery that will be done in September. It will be the final fix for her liver, but it will be much less involved that the surgery in April. Her gtube will stay in place for a full year. She needs stay pneumonia free, and make it through the next cold and flu season without problems and then we can pull her tube.
It two days we will move to a new home. We are leaving far more behind us than this house. We're leaving behind the nightmare of the past many years. Two really. We have had two years of hell on Earth - cancer, comas, frequent and long hospital stays - and we're ready to leave it all and start over. Praising God for the miracles he has brought to our family.
Today Angela had a swallow study. Let me give you a Reader's Digest version of the past year.
Angela has always had trouble swallowing. As an infant and toddler she had a tube because she couldn't swallow liquids without aspirating them into her lungs. Then around age 7..or maybe 10...she was diagnosed with cricopharngeal achalasia. Lets just say, it is a really bad swallowing disorder. At 14 or so it got much worse and we had to seek treatment in Boston. (we live in MN).
We had 19 years of weird medical problems, manic behavior, aggression, seizures, stroke, TIA's. All chalked up to "well this can be part of Down syndrome." We swallowed that pill and always moved forward. Angela was on a slew of antipsychotic medications.
Then in April 2015 she developed aspiration pneumonia. Then again in July...and September. We did a video swallow, stunned to find she had lost the ability to swallow ANYTHING safely. Absolutely everything went into her lungs, and solid foods stuck right in front of her airway. Her swallow was completely uncoordinated and dangerous for her. We were devastated, and telling her she couldn't eat anymore was beyond hard.
In October she had a tube placed. She entered the hospital her peppy, sassy self, and left on her very special formula depressed. She stopped smiling and started sleeping all the time. We called it depression until one day she wouldn't wake up. We found her on the floor of her room, face down in the carpet. I thought she was dead. She wasn't, she was in a coma.
And ambulance ride. Can't get an airway. Intubated. Weird IV line thingy jammed in her leg (what the hell was THAT? It looks weeks to heal!) 24 hours and she was awake and smiling. Nobody knew why, but I suspected it was some kind of reaction to that amazing formula we put her on.
Two weeks. Another coma. Lots of head scratching and even some finger pointing (Maybe we drugged her?) I refused to take her home until they figured it out. I told them to give her that formula, there was a problem, I knew it. A call to metabolic specialists lead to the first ammonia test, which was the super fast ticket to the Intensive Care Unit and a rescue drug that would clear the ammonia from her blood....at the risk of killing her kidneys.
A whole new life of protein restriction because her liver couldn't process it. Crazy formula and drugs to the tune of $30,000 per month. Potential rare disease diagnosis (OTC deficiency) while we waited for the necessary genetic testing. More ICU stays. More confusion. No longer responding to the rescue drug. One smart doctor who never stopped digging, on a whim ordered a liver scan.
There it was. Abernathy Type II malformation. The rarest of rare liver malformations. Blood not going where it needed to go so the liver couldn't do its job. A move to a different hospital who would know what to do. They sent us home while they researched a possible fix. Consulting doctors all over the country to come up with a plan.
April 8th. An experimental procedure they've never tried before. It worked!!!! She is a whole new person!! Manic moods gone. Aggression gone. Seizures gone. Happy. Always smiling. A totally new person. Maybe her swallow was better too? Ammonia affects muscle tone and control, maybe it was the cause of the swallowing problems? It was a long shot. We started letting her eat more. Drinking liquids. She was doing it without developing pneumonias. No sputtering. No choking.
And then today. A new swallow study. Truly stunned.
She passed.
100% oral eating as of today. She is whole again.
She's not done. She has one more surgery that will be done in September. It will be the final fix for her liver, but it will be much less involved that the surgery in April. Her gtube will stay in place for a full year. She needs stay pneumonia free, and make it through the next cold and flu season without problems and then we can pull her tube.
It two days we will move to a new home. We are leaving far more behind us than this house. We're leaving behind the nightmare of the past many years. Two really. We have had two years of hell on Earth - cancer, comas, frequent and long hospital stays - and we're ready to leave it all and start over. Praising God for the miracles he has brought to our family.
3 comments:
Oh my! I hadn't heard everything you've been through and I read part of this without breathing! Oh my word. But now, it seems... resolved?! Praise God! I can see why you're excited to begin a new chapter. What a journey you've all been on but now you've turned a corner and a whole new life begins before you. I'm SO excited for you all!! This post absolutely made my day!!
That is so awesome!!! I think there should be a parade or fire works or something else to mark this momentous occasion. You all must feel like you have been set free. Praise God, for Dr's who don't give up. Angela would not be where she is today with out you, you are the most amazing advocate for her. It is largely due to you standing firm on your gut feel that something isn't right, that has you coming out the other side to freedom. So, so happy for all of you! Xx
Hi! I've been reading you're blog for a long time, never commented till now. I'm so glad to hear Angela is finally doing well :-) Well I came out of my silent reader status to tell you it sounds like the needle they put in her leg was likely an IO needle. We use them in emergency settings when it isn't possible to quickly obtain normal IV access, to give people life saving medication (such as those needed to help with intubation.) As paramedics we are taught that the IOs don't have additional complication rates from normal IVs, it's interesting to read that Angela's took so long to heal, but from what you've written about her, it sound like if there is a chance of something going funny she'll usually find it unfortunately.
Good luck with your move, they can be stressful enough as a single person, I can't imagine figuring it out with so many children. Congratulations to Angela on being able to eat her favorite foods again!
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