Austin has chronic lung disease. Flu season and winter can be brutal for his respiratory system. But we have a secret weapon!
We have this sign on his stroller and diaper bag:
When out and about everywhere from Disneyland to doctor's offices other parents inquire where I got it from. Our sign came from the makers of Synagis (RSV medication), but you can order them here. You can also read more about RSV and who is at risk on our blog.
Children's Craniofacial Association has declared September as Craniofacial Acceptance Month.
HE'S MY SON
Yes, I am aware that September is over. But I can honestly say acceptance is something I fight for every day of the year, on behalf of my son. Born at 36 weeks, Austin was diagnosed with Branchiootorenal Syndrome, a rare genetic disorder that affects 1 in 40,000 people. BOR itself is not a craniofacial disorder it is an autosomal dominant genetic disorder that includes malformations of the ear and cysts in the neck, hearing loss, and malformations of the kidney.
As a result of BOR Syndrome, Austin's list of physical challenges also double as medical dictionary tongue twisters: Gastro-esophageal reflux disease, Microtia, Aural Atresia, Hemifacial Microsomia, Macrocephaly, Hypotonia and Tracheotomy, to name a few. These days I rattle them off with ease, I can explain what each one means to ME and how it affects Austin. BOR contributed to Austin's ears not developing, his jaw being so small that his tongue obstructed his airway, and some facial nerve paralysis.
Although Austin’s life would not be possible without a lot of assistive medical technology; he does have a life, a very full life indeed. He is like any typical 23 month old, he enjoys hanging out with his cousins at Disneyland, watches Blue's Clues, plays with blocks & puzzles and reads his favorite books.
While going for doctor visits or having therapists and nurses in his home are common for him, he remains friendly and (mostly) happy to see them. To explain how he lives his life with a Trach, G-tube or Chronic Lung Disease would require a much lengthier story than we have time and space for here, but in all of his struggles Austin has continued surprise us with his utter resilience. You can read the details on his blog.
HE HAS WHAT?
Microtia is an incompletely formed ear. It may be just a small ear, or other variations including having only a bump of tissue at the location where the ear should normally be found. Microtia may occur as an isolated deformity although it typically presents as part of a spectrum of other defects, either minor or major. It occurs more commonly in males and on the right side (unilateral). Approximately 10% may occur on both sides (bilateral) which is Austin’s case.
Aural Atresia is the closing or absence of an ear canal in the middle ear. Microtia and Atresia can occur alone or together. They can also be associated with Hemifacial Microsomia.
Children born with bilateral Microtia/Atresia often require bone-conduction hearing aids within the first few months of life. Austin got his hearing aid at 4.5 months. Although the surgeries to correct this problem used to be started as early as 4 years of age in the past, most surgeons prefer to start the external ear surgeries at about 6 to 7 years of age.
Hemifacial Microsomia is a condition in which the lower half of one side of the face does not grow normally. The most obvious sign of this condition is a partially formed ear or total absence of an ear and partial facial paralysis. The syndrome varies in severity, but always includes the underdevelopment of the ear and the mandible. This is the second most common facial birth defect after clefts.
The jaw abnormality (micrognathia) in children with hemifacial microsomia may range from a small but normally shaped parts of the jaw bone resulting in a mild asymmetry to complete absence of these structures resulting in a more severe jaw deformity. Because of the jaw abnormality these infants may be at risk for breathing and feeding problems and need to be evaluated by a specialist if there are any indications of airway compromise or failure to gain weight. Occasionally a tracheotomy and/or gastrostomy are needed to help with breathing and feeding.
Branchiootorenal (BOR Syndrome): Just like any other syndrome there is not one clear cut case of BOR, it is actually now classified as a spectrum disorder.
The B in BOR refers to the branchial arches, the area of the embryo that develops into the outer and middle ear, the neck and the lower part of the baby's face. There are several types of malformations of the branchial arches in BOR; Austin has auricular pits, which are very small holes about the size of the hole in a pierced ear, just below his ears. Normally these might be found on the neck area.
Oto refers to the ear and in particular the hearing loss that is part of the syndrome. The hearing loss can be sensorineural, conductive or mixed. It can be stable or progressive and the severity can range from mild to profound. Austin has bilateral, severe conductive hearing impairment (70-90dB).
Renal refers to the kidneys which can be abnormal in size, shape and/or structure. They may be smaller than usual or have a malformation that does not interfere with function or cause any symptoms. Austin thankfully does not have any kidney problems at present.
BEYOND THE FACE IS A HEART
Children with craniofacial differences come in many packages, but the one thing all have in common is that there is a living, breathing, feeling person behind those big named syndromes and disorders. These kids are funny, serious, loving, quirky, accepting and enduring. Each should be acknowledged for their own individual talents, as well as, their challenges.
Keep in mind beyond the face is a heart; the person you are staring, gawking or pointing at is someone's much loved child, it may be my son. Consider instead, a smile and a wave.
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There are many wonderful organizations you can consider supporting. They offer affected families medical treatment or support and resources.
In 1993 I had just completed my foster parent training. Little did I know that just a few minutes from my home a sweet baby was being born. That baby would soon become my son.
Jophie was born April 9, 1993. I received my foster parent license in the mail on May the 8th and on May the 9th just one month after his birth I received that call.
At 26 I was very nervous to be going to "just peek" at this sweet but very ill baby boy. This would be my first foster child and a baby at that! The next 30 minutes driving towards the hospital seemed like an eternity.
Brain damaged
Vegetable
Medically Fragile
CT Scans show sporadic damage
Guarded Life Expectancy
That was just a few of the words I would hear that day.
Did I hear those words?
Yes.....Sort of.
My eyes could not get past that beautiful baby lying there. His eyes and that hair....Oh my! There was so much more to him than all those big words and tests. He was small at just under 5 pounds. Small but mighty would soon outline the life of one baby boy who many thought had a grim future.
Yes! He was indeed about to show the world just how much more there was to him!
As we began our journey together, it became apparent to me that we were on no ordinary road to say the least. In the beginning the road was full of many bumps as we battled seizures, severe spasticity, feeding issues, many screaming sleepless nights and days as he battled drug and alcohol withdraws.
On February 11, 1997 just 2 months before his 5th birthday, I made this perfect little boy my son and so began our journey as mother and son with a much bumpier road full of its own share of curves, twists, and turns.
His health began to fail slowly but steadily as even more problems began to tumble into the mix. He began having numerous infections, pneumonia's, and rashes of unknown origin. The severity of his cerebral palsy and brain damage were becoming more evident with his age as he appeared to "stand still" in time cognitively. His motor skills were almost non-existent.
At this time it was determined he portrayed skills that of a newborn to 6 month old. Many tests and feeding studies were performed to check for reflux and aspiration as we felt he was doing both based on the chronic lung infections. The tests continued to prove non-conclusive.
By the age of 5 he became infected with varicella pneumonia from the chicken pox vaccine. YES it is unusual but it was what it was. I still thank God every day for my dear friend and sons pediatrician for listening to my heartfelt plea from his hospital room that early 3:00 a.m. morning. Even when the attending doctors felt I was wrong she believed me when I said, "He's dying before my very eyes". Sherries love for Jophie would prove to save his life that day. Thank you my dear friend!
Less than 6 hours later he was being rushed to the PICU in DIC(Disseminating Inter vascular Coagulation), shock as well as a severe case of chicken pox outside and within his lungs. Every breath would prove to rupture those blisters spreading it even more. His respiration's were in excess of 80, his heart rate was zooming. My baby was slipping away fast.
Papers were being shoved in my face. Sign this
Sign that
"Do you want us to save him?
"Is he a full code?"
Extraordinary measures taken?
Can we give blood if we need to?
Those were the questions being tossed at me as they gently guided me from his room.
One last kiss and one last "I love you" were what I thought would be my last.
Hours later by the grace of God AGAIN......Jophie was stable but very critical He would remain critical for several days which led into weeks and then months. This was by far one of the worst emotional roller coaster rides we had been on thus far.
Over the course of time he would prove to not just me but to many doctors, nurses, and onlookers just how strong a fighter he was and indeed how powerful our God really is. God moved mountains for Jophie and changed many lives during that hospital stay including mine.
The next few months things settled down a bit. He continued to battle chronic pneumonias, infections, and sometimes rare/odd super bugs however, we made the best of it and were able to make some wonderful memories!
By the time he was nearing 6 the pneumonias and infections were pretty much constant. Feeding was becoming more of an issue. Don't get me wrong Jophie LOVED food! His favorites were cotton candy yogurt, pintos and cheese from taco bell....(TONS of sour cream please!) :O) In fact I had just introduced him to chicken nuggets, cheese burgers, chili, and spaghetti! I had found a way to grind up just about anything with a combination of bread and apple sauce making it possible for him to eat.
Just beyond 6 years he was literally bombarded again with not one but 2 super bugs. Staph E. and enterobactor....UGH! By this time in his life, infectious disease had become our closest friends. Jophie continued to be named in their medical books for oddities and super bugs.(I would have rather he been anonymous).
Regardless, this hospital stay lasted nearly 6 months and once again he would meet face to face with death. The super bugs that he had were not only found in his blood but also in the cultures from his lungs. The only way for those to be there would be from aspiration. It was determined that testing be done again for the reflux and aspiration. The testing did indeed prove he was and most likely had been since the age of 2 ("Silently aspirating and refluxing") It had went undetected until that moment.
My world so I thought stopped. The one and only thing that Jophie and I could do together that was "normal" was eating and they were about to take that from us. I fought and wrestled with God. NO! I did not want this to happen. Don't take away the only "normal" thing we can do together. He enjoys eating so much!.
Finally, after tons of praying, struggling, fighting and arguing with God literally, I realized it was either this or he was going to die. My focus switched very quickly to being thankful for his life. I also knew that even though we had been dealt another blow, with my help Jophie would learn to adapt and we both would come out better and stronger than before!
All food at that time was stopped forever, a central line was placed, TONS of steroids were put on board for healing his lungs, many big gun antibiotics to battle these 2 bugs were being used AND TPN and Lipids were started to sustain him until which time he could recover enough for surgery.
The plan was to perform a fundoplication(Wrap the esophagus around itself to stop the refluxing and aspirating), place a feeding tube and cut the muscle where the intestines meet the stomach to create quicker dumping.
Without all the food and liquid that his little lungs had grown so accustomed to, his body slowly began to heal. When he showed what we felt was the best improvement, his surgery was scheduled. That 2 hour surgery turned into 12 because of many complications. The surgeon was able to perform all but one of the procedures. She was unable to cut the muscle for quicker dumping due to time spent under anesthesia, lack of access due to his misshapen back/chest, and scar tissue from damage sustained over the years.
Jophie healed slowly after surgery due to his puny state to begin with and by the first of the year we were able to return home. At home IV therapy was continued via his central line by me for the next 6 weeks. We had to continue for 6 more weeks after that because his blood was still testing positive for the staph E. Finally, all blood work came back negative and his central line was pulled.
During the summer/fall of 2004, Jophie would spend nearly 5 months hospitalized facing death toe to toe once again.
During this stay he would be infected with Proteus Morabilis TWICE, Pseudomonas, Stenotrophomonis Maltophilia, A pleural effusion and thank God it resolved itself, An AVM(Arterial Vascular Malformation) which caused a GI bleed and surgery was required to repair it, Paraflu(Which we both had for 2 weeks), Psueodomonis became active AGAIN, many allergic reactions to antibiotics, Tons of side effects, withdrawals, rashes, severe diarrhea, Neutropenia(Low white count), Pancytopenia(Low white count, red count, and platelets), blood transfusions, adrenal failure, AND finally home! Phew! What a Ride!
One year later he was still trying to recover. His body was battered, much weaker, and we continued to control break through seizures that at one time were mostly controlled and were unable to fully wean him from the very steroids that saved his life. We now fear his adrenals have failed because of it. We're hopeful the adrenals will begin working on their own again but, until that time the steroids that have caused this problem are once again saving his life.
Two years later in 2006 he would be hospitalized with Mycoplasma and C-Diff. Fortunately, I was able to bring him home after only a weeks stay. I along with Jophie's nurses would continue a 6 week long treatment regimen to kill those nasty bugs. It was during this time that I finally accepted the fact that I just couldn't keep track of all his medications. The many medications he was taking multiple times during the day made that abundantly clear. I usually manage just fine however, when he is ill you have to add the "extras" on TOP of what he already is taking and that's when my eyes begin to cross. The solution. A dry erase board mounted on my living room wall.:0)
March 2008 would prove to be another life altering hospital stay. After nearly going into respiratory failure, Jophie was transported via ambulance to the hospital where he landed in the PICU for the next 3 weeks in very critical condition.
Over the years Jophie's back/shape has gotten much worse making it next to impossible to treat him during a crisis. Fortunately, the wonderful PICU docs and staff were able to stabilize him on CPAP rather than placing him on the vent. He responded very well to this treatment and it would prove to save his life.
Tests would reveal an active Pseudomonas again. For those of you who haven't followed Jophie, he is colonized with Pseudomonas meaning it never goes away but rather goes into an inactive state. The plan is to keep those evil bug colonies down to a small number all the while trying to build up Jophie's good army who are in a constant battle to keep those evil ones at bay! I like to think of it as dragon slaying. Now if I could just find me a knight to slay these dragons we might be in business ;)
Because of Jophie's misshapen body and the poor state of his overall health it was determined that a trach was in order to prolong his life, give him a better quality as well as make it possible to effectively treat him during a crisis all of which I felt were very important if not life sustaining. What I didn't know was how long it was going to take to get to this point nor what he/we would have to endure along the way.
To say the last 7 months have been horrendous would be an understatement of great magnitude. If fact, 6 weeks into this new trach lifestyle I began questioning my decision to even allowing it.
The last 6 months Jophie has struggled with severe pain. He was coughing up blood on a regular basis. He was unable to sit up or to vocalize nor were we able to explain to him why. He's dealt with fear and frustration over that inability to vocalize. There has been lots of crying and gnashing of teeth. We both have had many sleepless nights and days as well as many trips to the ENT who was overseeing his progress as well as troubleshooting the problems. My battles with his insurance over yet more medical supplies related to this new trach continue as they still are limiting or refusing to cover needed/life sustaining items. The battles are never ending. Like I said we need a knight to slay these dragons who seem to come in many shapes and forms ;)
During the course of all this mess we were able to determine that Jophie was unable to wear the size or brand of trach that was originally placed. In fact it caused a huge ulcer and nearly bore a hole through his windpipe thus the cause of all the bleeding/pain/infections which led to a 2nd trip via ambulance due to his airway nearly closing off while Jophie's nurse and I watched helplessly. Ugh! A smaller pediatric trach was placed in the ER then he was admitted for surgery number 2 where the new pediatric trach would be evaluated as to whether or not to keep it and repair any damage sustained by the old trach as well as remove any granulation tissue that may have formed. To make matters worse, were having to boil/reuse these trachs which was causing the reoccurring infections not to mention warping the trachs which also caused more damage/injury and could have easily broken off in his airway. Fast forward to now October 2008.
Jophie is a much happier little guy! After many trials and lots of tears, we've found the brand of trach Jophie can use. Of course its custom and pricier than the rest but, its working and I for one am NOT going to muck with it! Would you? :0) His anxiety/fear has lessened considerably if not completely during daily trach cleaning as well as his weekly trach changes. He's been able to sit up for short periods of time and often for a couple hours. We've really seen improvements just in the last 3 to 4 weeks and have actually been able to do some "fun" things with him for the first time in sooooo long! I can't tell you how healing that has been for not only Jophie but me as well. He's still swollen quite a bit from the massive steroids but we are successfully weaning trying ever so hard to get him back to his maintenance dose. If all goes well barring any complications he should be back to maintenance by the second week of December! Phew! It's been a long road on this wean starting way back in march but so far so good!
Daily we continue to juggle steroid dosages by increasing and decreasing based on illness, stress, infections etc...Infections and super bugs continue to plague him. He now sees a pediatric endocrinologist at Columbus Childrens Hospital for his failing/failed adrenals. 5 years ago I changed his formula to Novasource pulmonary which is geared towards children with breathing and respiratory problems. This formula has absolutely been miraculous unfortunately we've just found out they will no longer be making it so the hunt is on for something comparable. I've also incorporated 3 different types of yogurt including an immunity booster as well as crystal light immunity which we add daily to his water infusions. Our hope is these things will help his compromised immune system as well as the candida that plagues him off and on. He now weighs around 100 pounds and daily we are slowly seeing the sparkle return to his eyes. I know without a doubt the new things we are trying are helping but the real credit goes to our heavenly father who continues to keep Jophie wrapped in his loving/healing arms.
The years have indeed been hard on Jophie. He has endured more than most do in an adult life thats for certain. He's faced death on more occasions than I can count. He's battled the ventilator on more than one occasion and he's had his share of surgeries and surgical procedures. He also has endured and continues to endure more tests, painful and unpleasant procedures, braces and corrective equipment, treatments, surgeries, rare and deadly super bugs and faces many monsters beyond what any of our imaginations could fathom. Jophie accepts all these daily routines that help him to survive and yes....he does this with a SMILE!
As you can see Jophie is MY LIFE. He is in every single breath I breathe. I love him so much sometimes it hurts which only fuels me further to fight for what he needs. He is indeed a special little guy who continues to touch lives everywhere he goes. I've been witness to miracles beyond measure and watched Doctors and Nurses stand with mouths gaping open as they try to comprehend what has just occurred. Time and Time again he survives, proclaiming loudly for all who see...
There is a God, I am loved, and above all he is in control. I am not a mistake nor am I damaged goods. I'm perfectly made and worthy of saving.
A masterpiece.
If he does nothing else in this world except for changing hearts and making others see beyond his disabilities and really SEE HIM as the masterpiece that he is, THEN he's accomplished everything.
It's been a long, scary road. We continue to travel this road together. Jophie and I with Jesus holding our hands.
A hero is said to be someone who is distinguished by exceptional courage and strength.
My life has forever been changed by such a person.
He's my hero
He's my son.
A Day in our Life
While most people have long since settled for a good nights sleep, Jophie and I are just beginning our day bright and early at 1 a.m.
1 a.m.--Reposition Jophie/Change diaper and bed pad and/or sheets that are usually wet/Give Carafate/Sterlize water/Add Immunity booster to his water infusion/Begin water infusion which takes 2 hours/Set Timer to alarm every 20 minutes/Albuterol Breathing Treatment
3 a.m.--Prepare Novasource Pulmonary for tube feeding/Crush Medications=Klonopin/Tegretol/Cortef/Bendaryl/Zofran/Mix crushed meds with sterile water and leave to soak/Repostion Jophie/Change diaper and bed pad and/or sheets that are usually wet/Give meds via Mic-key and begin Tube feeding/Set Timer to alarm every 20 minutes to give slow bolus till all feeding is done which takes around 3 hours
6 a.m.--Tube feeding is finished/Reposition Jophie/Change diaper and bed pad and/or sheets that are usually wet/Aluterol Breathing Treatment/Flovent/Begin water infusion which takes 2 hours/Set Timer to alarm every 20 minutes
7 a.m.--Carafate
7 a.m. to 9 a.m./Try to catch a few Zzzzzs!
9 a.m.--Nurse arrives/Prepare Novasource Pulmonary for tube feeding/Crush Medications=Klonopin/Tegretol/Cortef/Zofran/Prevacid/Mix crushed meds with sterile water and leave to soak/Reposition Jophie/Change Diaper and bed pad and/or sheets that are usually wet/Give Albuterol and Intal Breathing treatment/Give Flovent/Set Timer to alarm every 20 minutes to give slow bolus till all feeding is done which takes around 3 hours/I lie down to try and catch a couple more hours of sleep
12 p.m.--Reposition Jophie/Change diaper and bed pad and/or sheets that are usually wet
1 p.m.--Begin water infusion which takes 2 hours/Set Timer to alarm every 20 minutes/Give Carafate
3 p.m.--Prepare Novasource Pulmonary for tube feeding/Crush Medications=Klonopin/Tegretol/Cortef/Mix crushed meds with sterile water and leave to soak/Reposition Jophie/Change Diaper and bed pad and/or sheets that are usually wet/Give Abuterol Breathing Treatment/Give meds via Mic-key and begin Tube feeding/Set Timer to alarm every 20 minutes to give slow bolus till all feeding is done which takes around 3 hours/Eeek! I haven't eaten yet! Make a mad dash to grab some food!/Grab any meds at pharmacy/groceries/pay any bills that needs paying while I'm out and about
5 p.m. on Monday/Tuesday/Thursday Nurse leaves/3 p.m. on Wednesday/Friday Nurse leaves
6 p.m.--Reposition Jophie/Change Diaper and bed pad and/or sheets that are usually wet
7 p.m.--Give Albuterol and Intal Breathing Treatment/Give Carafate/Begin water infusion which takes 2 hours/Set Timer to alarm every 20 minutes
Try to snatch a few more Zzzzzs here!
9 p.m.--Prepare Novasource Pulmonary for tube feeding/Crush Medications=Klonopin/Tegretol/Cortef/Mix crushed meds with sterile water and leave to soak/Reposition Jophie/Change Diaper and bed pad and/or sheets that are usually wet/Give Abuterol Breathing Treatment/Give meds via Mic-key and begin Tube feeding/Set Timer to alarm every 20 minutes to give slow bolus till all feeding is done which takes around 3 hours
1 a.m. and we begin all over again!
This breakdown of our schedule doesn't even include the many other things that have to be done daily with no set time. I've only included the necessary things that HAVE to be done and HAVE to be on a schedule.
Now for the "other" things that also need tending to but we have to just work in between everything else
1. Tubing changes on his Oxygen Concentrator/Nebulizer/Air Compressor and Tubing to his trach
2. Equipment check on all of his equipment to make sure everything is in working order
3. Answering any alarms from those machines
4. Suctioning
5. Range of Motion/Stretching
6. Bathing/Grooming
7. Cleaning Filters to all machines
8. Sterlizing water and/or any equipment supplies that need it
9. Laundry and just keeping up with his Bed pads/sheets/clothes that tend to be soggy from just bout every body secretion you can imagine is a chore in itself and then theres mine!
10. Dishes
11. Sterlize/wash all his tubes/syringes/feeding bolus sets/Trach mask/Nebulizers masks/Trachs/bottles
12. Pooey Patrol! Every 3 days we have to give prophylatic medications to keep up with that little sluggish system of his! ;0)
13. Phone calls daily to Medical supply companies/Insurance
14. Restocking Medical Supplies/Putting Medical supplies away as they are delivered
15. Bills that have to be paid
14. Trips to the pharmacy for medications which seems to be neverending
16. The house and outside yard comes very last but still houses need cleaned and yards need mowing
Now try slipping in there an "outing" or something "fun" for Jophie besides all the medical stuff or how bout a doctors appointment or worse still he gets sick and then we have a whole nother set of meds and treatments!
Phew! Jophie sure is a busy kid and keeps his momma and nurses busy too! It definitely takes a team to care for him and meet all his needs on a daily basis. I'm very thankful for the nurses we have and wouldn't trade them for anything. In fact, they are more like family than "hired help". I like it that way especially for Jophie. Everything in his life is so "clinical/medical" and his home is one place I won't have that. He needs to feel comfortably in his home and know this is a fun/safe place to be! Now, if I could just find another nurse to fill these gaps we are having on the weekends I'd be one happy camper. We are still winging it alone every other Saturday and are limited 3 out of 4 of our Sundays. This weekend is one of those weekends and I for one can tell you....I am one tired momma! No regrets and no complaints from this corner though! I'd go to the ends of the earth to meet Jophies needs no matter how tired it makes me!
Meals 4 Wheels is a collaborative project inspired by Jophies need for transportation and medical supplies. Due to Jophies progressive illness, he is no longer able to ride in our current vehicle safely or comfortably. Our goal is not to get a new car, but to supply Jophie with a safe means of transportation. As medicaid continues to cut costs and pay for less of his medical supplies and equipment, Jophies needs have become greater. This is how the idea of the cookbook came about.
The actual cookbook was created from our success with weight loss. Since May of 2007, six of us collectively have lost a total of over 200 pounds. This was done exclusively by using the types of recipes in our cookbook, exercise, helpful tips as well as an overall lifestyle change. Through a lot of experimenting we were able to create healthful recipes without compromising what we all love most. The Taste! We believe that this cookbook can help you successfully lose weight and improve your own quality of life, just as by purchasing one you will help us to improve Jophie's quality of life.
Please support Trina and Jophie by leaving comments and checking out the cookbook site here.
