Dear Mr. Sparks,
John was diagnosed in utero by ultrasound with a congenital diaphragmatic hernia at 22 weeks gestation. This diagnosis has a 50-90% mortality rate. The amnio was fine. We were followed closely with monthly to twice weekly sonograms and daily kick counts. The concerns were his size (a little small) and (late in the pregnancy) decreased amniotic fluid. He arrived promptly on his due date in a delivery room at Stanford attended by his dad and filled with doctors, residents, interns and med students. He promptly cried and then peed on the OB. He was then whisked away, intubated and taken to NICU. It was a weekend, and the decision was made to wait for Monday morning to repair the hernia. (Most are repaired within 12-24 hours.) At 26 hours he decompensated. (2 hours prior to his scheduled surgery.) He had developed persistent pulmonary hypertension. Passing the criteria for ECMO (extra corporeal membrane oxygenation) by jumping up to a 80% chance of dying, he had his right carotid and jugular ligated for access to ECMO. (I tried, unsuccessfully, to get them to use any other access.) John remained on ECMO 5 days. He began to have lab work evidencing DIC and also acquired candida sepsis from the ECMO circuit. He was also noted to have a grade 1 right parietal bleed. He was taken off ECMO and was fairly stable. No fungal balls were noted in his eyes or his kidney. (This was good as the treatment would be enucleation or removal of a kidney. John's kidneys are fused at the top. He only has one "horseshoe" kidney.) He was given Amphoteracin for the candida sepsis. This promptly shut his kidney down. He was started on peritoneal dialysis. He was kept anesthetized and paralyzed (fentanyl drip and pavulon/tracrium). He remained on peritoneal dialysis for two months, interrupted briefly when he got peritonitis. For that he was given Gent and Vanco, resulting in his hearing loss. The decision was made to proceed with the CDH repair. Either his kidneys would work or they wouldn't. I wanted him awake before surgery. So he was weaned off the Fentanyl and (surprise!) his kidneys started to work. At 3 months the repair was done. At that time his left lung was just a bud. A few days post-op, the lung expanded fully. (He was the x-ray case of the day!) At 3 1/2 months he was weaned off the vent and nursed for the first time. At 4 months we went home. Seven days later we were back in the PICU for resp. failure. This started a cycle. In the hospital, intubated and paralyzed for 3-7 days, every 10 days for 6 weeks. Then I called a PICU Fellow and begged him to admit John before he crumpled again. He was finally diagnosed with subglottic stenosis. (Before that they told me he was getting viruses every 10 days.) The recommended treatment for the stenosis was every 2 week laser surgeries. Sometimes it was same day surgery and sometimes we had to stay for a week of paralytics and ventilation. Between surgeries I had John evaluated at our Regional Center and began OT/PT/Group. I also got him his first pair of hearing aids in June of '90. He got his first baby teeth and began to feed himself teething biscuits and bananas. (Refusing the bottle or cup, he continued to nurse till he was 14 months.) I realized that the lasers were not working, did research in the Stanford library and requested a consult from an ENT specialist at Childrens hospital in San Diego. In July (after a total of 9 lasers), we flew to San Diego for a double surgical evaluation. John spent another week paralyzed on a vent and was discharged with the additional diagnosis of reflux. In August John was trached to let the scarring in his airway mature (so he could get the cricoid split), and placed on meds for reflux. A diagnosis of esophageal ulcers was also made. For the next 11 months we went everywhere with a spare trach, ambu bag and suction machine. People followed us around the grocery store & asked questions. It was heaven having John home for 3 months at a time. During those 11 months, John learned to lift his head, pull to sit and then stand & walk with help. (Not bad for a child I was told would never use his hands or walk!) With John unable to vocalize, we began using more sign language. John caught RSV pneumonia and was rehospitalized from January through February '91. I took him home on oxygen with the new diagnosis of Asthma. (A nice addition to the anaphylactic reactions to milk protein and egg whites.) We added more meds and an O2 tank to the other equipment in John's diaper bag. We continued to see Dr Seid for every 3 month bronchoscopies to evaluate the progress of the subglottic scar tissue maturation. The doctors realized that with the continuing spray of stomach acids into his airway, this was not progressing. In May of '91, in another 6 hour surgery, John had a Nissan fundoplication and had a G tube placed. (By this time John was weaned off the O2.) I wanted John to continue to be able to eat by mouth, so I didn't use the G tube after the first week (except to burp John on 2 occasions). Since I was 'showing' quite a bit by this time, Dr Seid asked my due date. Then he said "let's get rid of the trach before then!" June 24 John had his airway rebuilt: an anterior-posterior cricoid split with rib grafts. In July, while bathing John, his g tube fell out. So we left it out. On my wedding anniversary in July '91, John and I flew home with NO trach, NO O2, NO suction machine and NO air compressor next to the crib. On 8/13/91 I went into labor. John, even then, wanted to steal the limelight from his sister. He had a respiratory arrest in the car. I did CPR for about 1 minute and he came right back. Kelsi (not wanting to be outdone by her brother) promptly was born in the backseat while dad (wanting to share the 15 seconds of fame) got a speeding ticket. John was discharged home in my care that day because Kelsi (mec stained from my adrenaline surge) was intubated (no mec below the cords) and had a lung blown (oh well!) while they observed her on the vent overnight. She wound up hospitalized for 10 days. On 8/15 John rearrested. I witnessed it and immediately attempted CPR. He presented as an obstructed airway. I met with a lot of resistance trying to ventilate him, but I was able to. We called 911. He was taken to Stanford and admitted. At least I had my babies in the same room! The Stanford docs wanted to retrach John. I called Dr Seid and he arranged a critical care flight to San Diego and bronched John himself. A stitch had grown out some scar tissue in his airway. One snip to fix it. At that time he was one of the endoscopic cases of the day for a group of international GI docs. They said his fundoplication looked great. His airway also looked like a typical airway. On 8/27/91 we were all home together. During September and October, John had a few minor surgical procedures (PE tubes). In November he started the infant hearing impaired class. He also began equine facilitated physical therapy. In December, John developed a bowel obstruction. So Kelsi, John and I spent our first Christmas together in the hospital. By February '92, John was standing and walking freely by himself. He also gave up trying to lay on Kelsi while she nursed. He vocalized ha & zzz and loved books and to wave bye bye. In May John began to eat table foods again. (He had reverted to baby foods when he was trached.) In September '92 we were rehospitalized for another bowel obstruction. Then John had his first seizure. I finally quit bargaining with god. ("OK God, I can deal with this, but please not that.") I took John home refusing meds for an isolated seizure. At home, John was picking up and carrying things on command. In October, a repeat bowel obstruction with bowel twisted in the chest, necessitated another major (8 hour) surgery. They did a lysis of adhesions, appendectomy (the appendix was buried in scar tissue) and replaced the diaphragmatic patch. By December, John could pull on his pants, try to put on his shoes, sign "more" and "finished" and loved to play tag with his mom. Early in '93, John was doing simple puzzles, pulling a small chair to the kitchen to watch me cook and picking out cans of food for me to fix. He took Kelsi her bottle on command and without being asked. When Kelsi signed "shoes, shoes, where are my shoes" he took them to her. At this time I pulled John out of the county special ed. and taught him at home. I followed some of Lovaas (only positive reinforcement) and got help by hiring Grad students in psyche and speech therapy (both were 'signers'). When I finally found a good cross categorical class, I sent him to school with one of my Lovaas assistants. The school still insisted that John didn't qualify for OT or PT. So I got into the medical insurance and CCS games. We started paying for our own twice weekly PT/OT/speech. John began floating on his back in the tub and walking up stairs without holding a rail. In April of 1993, John began having frequent seizures. I insisted he stay off Dilantin and Phenobarb. We started on Tegretol. But the seizures increased every time the tegretol was increased. He kept metabolizing the tegretol faster so we couldn't get a therapeutic level. By June I insisted we change the medication. I also changed neurologists, as one said we would never control the seizures on meds, he needed a lobectomy. He also refused to write prescriptions for OT and PT saying that John suffered a massive stroke from the ECMO and it would not help anyway. My new neurologist put John on Felbatol. It was great in that it is not sedating. At that time, there was no maximum dose. (This was before felbamate was linked to aplastic anemia.) We just continued increasing the dose to resolution of seizures. I also obtained Valium to give John at the onset of the seizures (IV Valium given rectally with a small syringe (no needle). (There was nothing like the feeling of helplessness watching John seize for 20 minutes with no meds to give him. As an ICU nurse I was used to having some control, as well as meds on hand and protocols.) The seizures (always as he was falling asleep and beginning with the left foot, up the left side, before crossing & becoming complete) now only lasted as long as it took me to get the Valium (taped to the wall at the head of the bed). They never progressed beyond his left foot. The seizures decreased from 2-3 per week, to one every six months (while still on asthma meds) to one per year. We also switched from cross categorical classrooms to hearing impaired classrooms. Kelsi was starting dance and interested in gymnastics, so I began the kid activities. John enjoyed gymnastics (with me as interpreter and aide) and swimming. We no longer were involved with equine therapy and dropped PT when the therapist said that the gymnastics was helping John more than she could. I found a good oral-motor therapist for speech and we began to get some good approximations. (John is now water safe and loves the pool.) I had been looking through new drug books we were evaluating for the unit, and I noticed that John was on twice the maximum dose of felbamate. The major side effect listed for felbatol was alterations in thought processes. Despite warnings from John's neurologist that he would have massive seizures, I very slowly (over 3 months) weaned John off his felbamate by July 1997. No seizures! In September he was the first "full inclusion" child at our neighborhood school in Pacifica, CA. He learned the alphabet and to count to 10. He also learned to watch other children, and do some of what they do.
John has been in gymnastics (I have to give signed instructions, tell him to follow the kids, and sometimes demonstrate) and in swimming (I have to be in the water to sign and help the instructor), and can jump on a trampoline and walk on a balance beam and is water safe. John can ski the bunny slopes with 'toe clips' on his skis, but prefers to sled. In June of 1998 we went to team training and John received "Vinton," his canine companion for independence. Vinton has been teaching John responsibility and has helped socialize him. Vinton has also become a companion to John, and an anchor for John in airports. They play fetch and tag in the back yard. In August of '98, I sold our house in Pacifica, CA to move us to Charlotte to be with the kids dad (he had been transferred 2 years earlier, and was flying home on weekends to see the kids and watch them so I could work). We put John in the local hearing impaired multihandicapped classroom as he does not fit into a hearing impaired class. John's current medical status is stable. He outgrew his allergies to milk proteins and egg whites. He outgrew his asthma. John's last focal seizure was November '95. We will be drug free for 2 years this July. John has had febrile seizures (very different from his usual seizures) spring of '98 and '99. His current diagnoses are: Hearing impaired, auditory processing disorder, mild atypical CP, apraxia and severe oralmotor apraxia, "static encephalopathy" and history of seizure disorder. Also PDD-NOS. My diagnosis for him is medically and surgically traumatized. He had a total of 24 surgeries by 3.5. John enjoys riding his bike (one training wheel) mostly over off road terrain. He is recently attempting to use roller skates. His receptive language (when not under stress) using sign language is fairly good. Perhaps at a 3-4 year old level. His expressive sign language is at about a 2 year old level. Oral speech has deteriorated since our move. It took me 6 years to find the first great oral-motor specialist, and I haven't found one here yet. He can write the alphabet in upper and lower case letters. He learns 3 spelling words each week. He can count to 30 and has learned the days of the week. He remains a flight risk. He continues to find ways out of the locked house and yard. Our canine companion has found him for me on 2 occasions. He has a definite lack in attending and socializing skills. His auditory processing and oral-motor apraxia are his most limiting features at this time. I am trying hyperbaric oxygen this summer. I would like to see if it helps his auditory processing, attending and his learning difficulties. I probably have left something out. And I'm sure the story will continue.
Sincerely,
John's mom, Debra Hall (North Carolina)
John was diagnosed in utero by ultrasound with a congenital diaphragmatic hernia at 22 weeks gestation. This diagnosis has a 50-90% mortality rate. The amnio was fine. We were followed closely with monthly to twice weekly sonograms and daily kick counts. The concerns were his size (a little small) and (late in the pregnancy) decreased amniotic fluid. He arrived promptly on his due date in a delivery room at Stanford attended by his dad and filled with doctors, residents, interns and med students. He promptly cried and then peed on the OB. He was then whisked away, intubated and taken to NICU. It was a weekend, and the decision was made to wait for Monday morning to repair the hernia. (Most are repaired within 12-24 hours.) At 26 hours he decompensated. (2 hours prior to his scheduled surgery.) He had developed persistent pulmonary hypertension. Passing the criteria for ECMO (extra corporeal membrane oxygenation) by jumping up to a 80% chance of dying, he had his right carotid and jugular ligated for access to ECMO. (I tried, unsuccessfully, to get them to use any other access.) John remained on ECMO 5 days. He began to have lab work evidencing DIC and also acquired candida sepsis from the ECMO circuit. He was also noted to have a grade 1 right parietal bleed. He was taken off ECMO and was fairly stable. No fungal balls were noted in his eyes or his kidney. (This was good as the treatment would be enucleation or removal of a kidney. John's kidneys are fused at the top. He only has one "horseshoe" kidney.) He was given Amphoteracin for the candida sepsis. This promptly shut his kidney down. He was started on peritoneal dialysis. He was kept anesthetized and paralyzed (fentanyl drip and pavulon/tracrium). He remained on peritoneal dialysis for two months, interrupted briefly when he got peritonitis. For that he was given Gent and Vanco, resulting in his hearing loss. The decision was made to proceed with the CDH repair. Either his kidneys would work or they wouldn't. I wanted him awake before surgery. So he was weaned off the Fentanyl and (surprise!) his kidneys started to work. At 3 months the repair was done. At that time his left lung was just a bud. A few days post-op, the lung expanded fully. (He was the x-ray case of the day!) At 3 1/2 months he was weaned off the vent and nursed for the first time. At 4 months we went home. Seven days later we were back in the PICU for resp. failure. This started a cycle. In the hospital, intubated and paralyzed for 3-7 days, every 10 days for 6 weeks. Then I called a PICU Fellow and begged him to admit John before he crumpled again. He was finally diagnosed with subglottic stenosis. (Before that they told me he was getting viruses every 10 days.) The recommended treatment for the stenosis was every 2 week laser surgeries. Sometimes it was same day surgery and sometimes we had to stay for a week of paralytics and ventilation. Between surgeries I had John evaluated at our Regional Center and began OT/PT/Group. I also got him his first pair of hearing aids in June of '90. He got his first baby teeth and began to feed himself teething biscuits and bananas. (Refusing the bottle or cup, he continued to nurse till he was 14 months.) I realized that the lasers were not working, did research in the Stanford library and requested a consult from an ENT specialist at Childrens hospital in San Diego. In July (after a total of 9 lasers), we flew to San Diego for a double surgical evaluation. John spent another week paralyzed on a vent and was discharged with the additional diagnosis of reflux. In August John was trached to let the scarring in his airway mature (so he could get the cricoid split), and placed on meds for reflux. A diagnosis of esophageal ulcers was also made. For the next 11 months we went everywhere with a spare trach, ambu bag and suction machine. People followed us around the grocery store & asked questions. It was heaven having John home for 3 months at a time. During those 11 months, John learned to lift his head, pull to sit and then stand & walk with help. (Not bad for a child I was told would never use his hands or walk!) With John unable to vocalize, we began using more sign language. John caught RSV pneumonia and was rehospitalized from January through February '91. I took him home on oxygen with the new diagnosis of Asthma. (A nice addition to the anaphylactic reactions to milk protein and egg whites.) We added more meds and an O2 tank to the other equipment in John's diaper bag. We continued to see Dr Seid for every 3 month bronchoscopies to evaluate the progress of the subglottic scar tissue maturation. The doctors realized that with the continuing spray of stomach acids into his airway, this was not progressing. In May of '91, in another 6 hour surgery, John had a Nissan fundoplication and had a G tube placed. (By this time John was weaned off the O2.) I wanted John to continue to be able to eat by mouth, so I didn't use the G tube after the first week (except to burp John on 2 occasions). Since I was 'showing' quite a bit by this time, Dr Seid asked my due date. Then he said "let's get rid of the trach before then!" June 24 John had his airway rebuilt: an anterior-posterior cricoid split with rib grafts. In July, while bathing John, his g tube fell out. So we left it out. On my wedding anniversary in July '91, John and I flew home with NO trach, NO O2, NO suction machine and NO air compressor next to the crib. On 8/13/91 I went into labor. John, even then, wanted to steal the limelight from his sister. He had a respiratory arrest in the car. I did CPR for about 1 minute and he came right back. Kelsi (not wanting to be outdone by her brother) promptly was born in the backseat while dad (wanting to share the 15 seconds of fame) got a speeding ticket. John was discharged home in my care that day because Kelsi (mec stained from my adrenaline surge) was intubated (no mec below the cords) and had a lung blown (oh well!) while they observed her on the vent overnight. She wound up hospitalized for 10 days. On 8/15 John rearrested. I witnessed it and immediately attempted CPR. He presented as an obstructed airway. I met with a lot of resistance trying to ventilate him, but I was able to. We called 911. He was taken to Stanford and admitted. At least I had my babies in the same room! The Stanford docs wanted to retrach John. I called Dr Seid and he arranged a critical care flight to San Diego and bronched John himself. A stitch had grown out some scar tissue in his airway. One snip to fix it. At that time he was one of the endoscopic cases of the day for a group of international GI docs. They said his fundoplication looked great. His airway also looked like a typical airway. On 8/27/91 we were all home together. During September and October, John had a few minor surgical procedures (PE tubes). In November he started the infant hearing impaired class. He also began equine facilitated physical therapy. In December, John developed a bowel obstruction. So Kelsi, John and I spent our first Christmas together in the hospital. By February '92, John was standing and walking freely by himself. He also gave up trying to lay on Kelsi while she nursed. He vocalized ha & zzz and loved books and to wave bye bye. In May John began to eat table foods again. (He had reverted to baby foods when he was trached.) In September '92 we were rehospitalized for another bowel obstruction. Then John had his first seizure. I finally quit bargaining with god. ("OK God, I can deal with this, but please not that.") I took John home refusing meds for an isolated seizure. At home, John was picking up and carrying things on command. In October, a repeat bowel obstruction with bowel twisted in the chest, necessitated another major (8 hour) surgery. They did a lysis of adhesions, appendectomy (the appendix was buried in scar tissue) and replaced the diaphragmatic patch. By December, John could pull on his pants, try to put on his shoes, sign "more" and "finished" and loved to play tag with his mom. Early in '93, John was doing simple puzzles, pulling a small chair to the kitchen to watch me cook and picking out cans of food for me to fix. He took Kelsi her bottle on command and without being asked. When Kelsi signed "shoes, shoes, where are my shoes" he took them to her. At this time I pulled John out of the county special ed. and taught him at home. I followed some of Lovaas (only positive reinforcement) and got help by hiring Grad students in psyche and speech therapy (both were 'signers'). When I finally found a good cross categorical class, I sent him to school with one of my Lovaas assistants. The school still insisted that John didn't qualify for OT or PT. So I got into the medical insurance and CCS games. We started paying for our own twice weekly PT/OT/speech. John began floating on his back in the tub and walking up stairs without holding a rail. In April of 1993, John began having frequent seizures. I insisted he stay off Dilantin and Phenobarb. We started on Tegretol. But the seizures increased every time the tegretol was increased. He kept metabolizing the tegretol faster so we couldn't get a therapeutic level. By June I insisted we change the medication. I also changed neurologists, as one said we would never control the seizures on meds, he needed a lobectomy. He also refused to write prescriptions for OT and PT saying that John suffered a massive stroke from the ECMO and it would not help anyway. My new neurologist put John on Felbatol. It was great in that it is not sedating. At that time, there was no maximum dose. (This was before felbamate was linked to aplastic anemia.) We just continued increasing the dose to resolution of seizures. I also obtained Valium to give John at the onset of the seizures (IV Valium given rectally with a small syringe (no needle). (There was nothing like the feeling of helplessness watching John seize for 20 minutes with no meds to give him. As an ICU nurse I was used to having some control, as well as meds on hand and protocols.) The seizures (always as he was falling asleep and beginning with the left foot, up the left side, before crossing & becoming complete) now only lasted as long as it took me to get the Valium (taped to the wall at the head of the bed). They never progressed beyond his left foot. The seizures decreased from 2-3 per week, to one every six months (while still on asthma meds) to one per year. We also switched from cross categorical classrooms to hearing impaired classrooms. Kelsi was starting dance and interested in gymnastics, so I began the kid activities. John enjoyed gymnastics (with me as interpreter and aide) and swimming. We no longer were involved with equine therapy and dropped PT when the therapist said that the gymnastics was helping John more than she could. I found a good oral-motor therapist for speech and we began to get some good approximations. (John is now water safe and loves the pool.) I had been looking through new drug books we were evaluating for the unit, and I noticed that John was on twice the maximum dose of felbamate. The major side effect listed for felbatol was alterations in thought processes. Despite warnings from John's neurologist that he would have massive seizures, I very slowly (over 3 months) weaned John off his felbamate by July 1997. No seizures! In September he was the first "full inclusion" child at our neighborhood school in Pacifica, CA. He learned the alphabet and to count to 10. He also learned to watch other children, and do some of what they do.
John has been in gymnastics (I have to give signed instructions, tell him to follow the kids, and sometimes demonstrate) and in swimming (I have to be in the water to sign and help the instructor), and can jump on a trampoline and walk on a balance beam and is water safe. John can ski the bunny slopes with 'toe clips' on his skis, but prefers to sled. In June of 1998 we went to team training and John received "Vinton," his canine companion for independence. Vinton has been teaching John responsibility and has helped socialize him. Vinton has also become a companion to John, and an anchor for John in airports. They play fetch and tag in the back yard. In August of '98, I sold our house in Pacifica, CA to move us to Charlotte to be with the kids dad (he had been transferred 2 years earlier, and was flying home on weekends to see the kids and watch them so I could work). We put John in the local hearing impaired multihandicapped classroom as he does not fit into a hearing impaired class. John's current medical status is stable. He outgrew his allergies to milk proteins and egg whites. He outgrew his asthma. John's last focal seizure was November '95. We will be drug free for 2 years this July. John has had febrile seizures (very different from his usual seizures) spring of '98 and '99. His current diagnoses are: Hearing impaired, auditory processing disorder, mild atypical CP, apraxia and severe oralmotor apraxia, "static encephalopathy" and history of seizure disorder. Also PDD-NOS. My diagnosis for him is medically and surgically traumatized. He had a total of 24 surgeries by 3.5. John enjoys riding his bike (one training wheel) mostly over off road terrain. He is recently attempting to use roller skates. His receptive language (when not under stress) using sign language is fairly good. Perhaps at a 3-4 year old level. His expressive sign language is at about a 2 year old level. Oral speech has deteriorated since our move. It took me 6 years to find the first great oral-motor specialist, and I haven't found one here yet. He can write the alphabet in upper and lower case letters. He learns 3 spelling words each week. He can count to 30 and has learned the days of the week. He remains a flight risk. He continues to find ways out of the locked house and yard. Our canine companion has found him for me on 2 occasions. He has a definite lack in attending and socializing skills. His auditory processing and oral-motor apraxia are his most limiting features at this time. I am trying hyperbaric oxygen this summer. I would like to see if it helps his auditory processing, attending and his learning difficulties. I probably have left something out. And I'm sure the story will continue.
Sincerely,
John's mom, Debra Hall (North Carolina)
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