In April 1999, our 7 month old daughter Erin was diagnosed with Chiari Malformation (level 1). This article is a short chronicle of Erin's particular condition, an explanation of Chiari in general, and a diary of her progress.
NOTE: In March 2000, Erin was also diagnosed with Acute Lymphoblastic Leukemia (ALL), and in December 2000 she was diagnosed with William's Syndrome (a deformation of the 7th chromosome) The bottom portion of this page is also dedicated to documenting her battle with these diseases.
Chiari Malformation is the name associated with a condition in which the posterior fossa cavity (lower rear portion of the skull) is malformed and/or is not large enough for the cerebellum and cerebellar tonsils - which cover the cerebellum. Due to this lack of space, the cerebellar tonsils and cerebellum begin being squeezed like a water balloon through the base of the skull and down into the spinal column. This in turn squeezes the brainstem and also blocks the flow of Cerebrospinal Fluid (CSF).
Chiari malformation is seen in many levels of severity, and it in turn can cause other problems such as syringomyelia (a syrinx - fluid filled cyst - in the spinal canal) or hydrocephalus (complete blockage of fluid flow causing fluid build up in the brain) to name a few. The levels of severity are generally associated with the amount of displacement of the cerebellar tonsils down the spinal column. In extreme cases, the cerebellum itself, and the brain's fourth ventricle can become displaced.
Chiari can only be seen through MRI scans and is not usually seen (or diagnosed) in infants due to the low frequency of MRIs performed on infants as well as the inability of infants to communicate the symptoms.
Usually, Chiari is found in older children and adults after years of persistent symptoms. Reported symptoms can include:
- severe migraines
- reflux and vomiting
- problems swallowing
- respiratory problems
- central apnea
- balance problems
- numbness in limbs
According to many studies and reports, Chiari is often misdiagnosed as another illness due to the commonality of symptoms with other diseases. It is the combination of many of these symptoms which can lead to the discovery and diagnosis of Chiari.
Chiari is also present in children with diseases such as spina bifida and is a contributing factor to the symptoms seen in these children.
Birth to 3 months
As early as 3 days old, Erin displayed extreme colic-like symptoms, with several important differences. Our son had a severe case of colic, so we were familiar with it. Generally, with colic, symptoms included:
- inconsolable crying/screaming at a consistent period each night (4-6 hours)
- gastrointestinal-like problems (pulling knees into chest)
- curling up while crying
With Erin, initial symptoms included:
- inconsolable screaming during all waking periods - not just evening or fixed periods
- no more than 2-3 hours of sleep at a time
- poor feeding
- rigid body, arched back, arms extended, head back while screaming
- tugging at ears and hair
- chronic ear infections
We believed something was wrong right from the start.
During her first 3 months, Erin opened her eyes very little, and screamed as if in massive pain during most all waking moments. She nursed poorly. It was assumed she may be lactose-intolerant. During this period, she was removed from breast-feeding and went on 7-10 day "trial periods" each of several different types of formula (Soy, Hypo-allergenic, etc.). There was no change. She took many trips to the Pediatrician, but the diagnosis remained "probable colic and potential reflux". She was placed on Zantac for reflux.
On a personal note: It was the Holidays, and we were kicked out of every family function (her screams could clear a room fast). We tried every colic "snake-oil" we could find from homeopathic remedies to vibrating beds. Still no change. Riding in the car did not calm her down. We basically became shut-ins as we refused to drive anywhere more than 10 minutes away and no one would want to have her at their house. Home life became very strained.
4 to 5 months
At four months Erin opened her eyes more frequently and began to have periods (15 minutes at a time) of wakefulness without screaming or crying. She still did not sleep for more than 3 hours at a time. At this point colic should be waning, but Erin would have periods of up to 72 hours where she would not sleep more than 10-15 minutes at a time and would scream constantly, leaving her hoarse and us exhausted. At this time, she had upper and lower GI tests performed, as well as complete blood and urine tests looking for any potential problem. Only very minor reflux was found.
Erin was on a daily regimen of antibiotics (chronic ear infections), Zantac (for reflux), mylanta (for gas), laxatives (for constipation caused by the Zantac), Tylenol, and Levisin PB (Phena-barbatol). Nothing changed.
At five months, Erin began to develop a little more rapidly and had longer good wakeful periods and slightly longer sleeping periods (up to 5 hours some nights). But she still had more periods of inconsolable pain. It was at this time that a CT scan was ordered to begin looking at possible neurological causes. The CT scan came back showing a potential problem with the fourth ventricle, but the scan was cloudy and inconclusive. Based on this, an MRI was ordered.
On a personal note: During this period we endured a myriad of doctors, specialists and nurses who seemed to wonder why we were concerned with a "fussy" baby. We would have killed for "fussy" (and wanted to kill all those who called her condition "fussy"). We were even told once "you do know that all babies will cry sometimes." The amount of medical personnel who told us we were overreacting was incredible. As parents, we were both battling bouts of anxiety and depression, we argued constantly, and paid very little attention to our 3 year old son - who has been wonderfully patient during this ordeal.
To Lisa's credit (Erin's mom), she never gave up. No matter how much she was made to feel like a bad parent who simply didn't know how to calm her "slightly fussy" child, she persisted (Get the idea we have a pet peeve now about the word "fussy"?). And to our pediatrician's credit (Dr. Joseph Bailey), he listened and persisted as well.
6 to 7 months
At six months, the MRI was performed, and Erin had tubes put in her ears. She had already sustained slight hearing loss in both ears. The fourth ventricle appeared normal on the MRI, but the Chiari malformation was diagnosed. We had never heard of Chiari at that time.
Below is a picture from Erin's MRI showing her condition.
After the diagnosis, we were referred to Dr. Kerry Crone, Head of Pediatric Neurosurgery at Children's Hospital in Cincinnati. We have since come to find that he is one of the finest pediatric neurosurgeons in the country, and people come from all around the world to see him. We are fortunate to have him here in our backyard.
As we read everything we could find on Chiari, Dr. Crone ordered Barium swallowing tests and a sleep study be performed. In his view, while the MRI distinctly shows the Chiari malformation, he is opposed to surgery unless there are tangible signs that Chiari is in fact causing problems. Because of the pressure on the brainstem, a tangible sign would be loss of involuntary motor skills (swallowing and/or breathing).
At eight months Erin took the Barium swallow test and the sleep test. She passed the swallow test with good results. However her sleep test showed central sleep apnea, with loss of respiration for periods of up to 12 seconds which included a drop in oxygen level. Dr. Crone believed that was the evidence needed and he recommended decompression surgery within 4 weeks. Erin was also placed on an apnea monitor.
This diagnosis and treatment were confirmed by Dr. Mark Luciano (Head of Pediatric Neurosurgery, The Cleveland Clinic).
During this same period, Erin began having more episodes of pain and she began spitting up more after eating. She was now sleeping about four to five hours at night but had an extremely hard time getting to sleep. She had good wakeful periods up to an hour at a time. However, certain developmental factors seemed to be delayed.
At eight months she:
- could not sit up for more than a few minutes
- was very unstable sitting
- shakes when sitting or reaching
- can not crawl
- can not roll from belly to back
- cannot hold her bottle
- does not "talk" - meaning making sounds or cooing (although she laughs)
We were given no guarantees that all of these problems were related to the Chiari, but only told that it was possible some of them may be affected by it.
Just prior to surgery Erin's condition seemed to be getting worse (maybe we were just looking for it?). While she had passed the swallow test with no problems five weeks prior, she was now beginning to gag slightly when fed and she was spitting up quite a lot after she ate. No full vomiting, but she would spit up about 25% of her intake over the course of an hour.
Her apnea monitor still had not gone off, which was good, but she was starting to sleep less, wake up more agitated, and not go down for naps.
Her "fits of pain" also seemed slightly increased. She would cry out suddenly and grab her ears, her hair or the back of her head and scream for 10-30 seconds - then go back to "normal".
The night before her surgery, Erin only slept one hour and was not happy that she couldn't eat.
"Normal" surgery for Chiari level 1 consists of a posterior fossa decompression, a lamenectomy, opening of the dura, and reduction of the cerebellar tonsils. Also, if conditions such as syringomyelia or hydrocephalus exist, a shunt may need to be installed.
In layman's terms, this all means that the opening at the base of the skull is enlarged, the rear portion of the C1 (and possibly C2) vertebrae is removed, the membrane covering the brain inside the skull (the dura) has a section removed, and the cerebellar tonsils themselves are "trimmed" down.
To see a pictorial description, see the National Fibromyalgia Research Association's Chiari page.
Dr. Crone is one of the few in the world who doesn't always go through the dura and reduce the tonsils. His procedure is to perform the decompression and the lamenectomy and then perform an ultrasound on the dura to see of the patient has already received adequate pressure relief and to see if full CSF flow has been restored. If not, then he continues through the dura - which is later patched with cow's heart tissue.
In Erin's case, she was lucky. Her Level 1 Chiari consisted of a 9mm displacement below the skull but she had neither a syrinx nor hydrocephalus. This was great news because they can cause other serious complications and symptoms. So it was anticipated that only the "normal" procedure would be done.
Erin arrived at the hospital at 6 a.m. and was in the OR by 10:30. Her operation lasted only about 1 hour and 30 minutes, but overall time was about 3 hours because they had a problem with her arterial IV and had to stop the operation and re-prep her.
In our post-op consultation, Dr. Crone told us that after enlarging the hole in her skull she appeared to have complete fluid flow restoration around the brainstem. Her skull, he said, had been 4 times as thick as it should have been - which obviously contributed to the condition (so, she really is thick-headed!). Due to this, and due to her age and development, he chose not to go through the dura and reduce the tonsils. The downside to this is that it may take longer to truly see the results of the surgery because the tonsils don't immediately move back up out of the spinal column. He told us that the skull malformation (thickness) was due to a chromosome defect, but that it was not a concern for bone formations elsewhere in her body. We'll need to follow up on this and report details back later.
Erin was strong and there were no complications during surgery (other than the problem with the IV). Also, because the dura was not penetrated, many post-op risk factors were greatly reduced - such as:
- bacterial meningitis caused by the surgery
- physical brain damage
- neurological damage
By 1:30 p.m. she was breathing better than expected and had her breathing tube removed. She was moved to the pediatric ICU (intensive care unit) by 2:00 p.m.
In this Post-op section, we will have a running log of Erin's recovery from day 1 through at least her first follow-up. Please note, all our observations are very subjective, and we're trying not to be overly optimistic - which is difficult considering the course of the past 9 months.
These "highlights" will be filled in with greater details when time allows.
Day 1 - Thursday: (The day of surgery)
Very bad day for Erin. Excruciating pain and little pain relief for over 10 hours. Morphine had an adverse effect - caused severe itching and irritability. Finally sedated 11 hours after surgery with Adavan and Chlorohydrate - risk of respiration failure. Sedated for 6 hours overnight.
Slightly better day; less screaming; still no happiness though. Medication changed from morphine to Fentanyl (self-dosed by us through IV). This seemed to help. Erin ripped one of her IVs out - very messy. Had another IV put in - very painful. Moved from ICU to pediatric surgery recovery ward.
Better day. Erin had periods of wakefulness without screaming. However, she appeared glassy-eyed most of the time. Her neurological checks were all fine though. She was check every 2 hours, and she still received doses of Fentanyl about every hour, along with Tylenol every 4 hours. Still taking Zantac. Given an anti-inflammatory, even though her swelling appeared normal.
Unbelievable turnaround in just one day. Erin is sitting up, smiling, very alert and playful. No medicine except Tylenol for over 6 hours, so we removed the IV and the Fentanyl. She was never so happy as to have that IV out. Appetite is improving and she's eating solid foods (baby food) again. Tylenol reduced to every 6 hours.
Unexpectedly, Erin is released from the Hospital. As close as two days ago doctors thought she'd be here for at least two more days from today. She's acting normal, no signs of real pain, happy and alert. Risks of infection and Meningitis all but gone. Erin slept for 7 hours this night - a new record for her. But she's tired so we're trying not to read much into it.
Not as good a day today. Erin was very crabby (but still much better than before) and didn't want to sleep or take naps. But she is eating so much better, and no spitting up. She sits playing by herself for longer periods of time. but she was very tired today.
If every day could be like today, we'd think she was cured for sure. Erin was pretty much a happy camper all day. She only got fussy when she was tired or hungry - like it should be. She ate well, napped well, and had a great disposition. She slept another 7 hours last night. She did however grab her head and/or ears a few times today like she used to - but it was only when she was tired.
Okay, so her days are on-again off-again. Pretty much a day like Day 6 today. She was in a pretty good mood for part of the day, but didn't want to nap or go to sleep at night. She also spit up some today and grabbed her head a few times. So, again we're reminding ourselves to be cautious about thinking everything's cured. But overall, she still seems so much different than before.
True to form, this is Happy Camper day. A tiny bit of spit-up, but she's eating well and sleeping well and generally happy. She also appears to be more balanced than she was before - not as unsteady when leaning over or sitting.
Still pretty happy today. The effects of surgery are all but gone, and her incision is healing great. With nothing big to report, it seems it's now time to start watching her development over a given period of time (week or month) rather than day to day. So this is it for a while.
Day 30 (week 4)
Erin has been developing tremendously. Sitting up, rolling, pulling herself to standing, talking (okay "babbling" is more like it), having great periods of wakefulness, and sleeping! She has consistently been sleeping anywhere from 7-10 hours a night and she can put herself back to sleep most times when she wakes up. She is a completely different child.
For several days we were concerned because she has been spitting up more often recently. However, a consultation with our Pediatrician today assured us that it is most likely because she is now so active (sitting and rolling) and subsequently compressing her stomach when she moves. Not a relapse. She has no other signs of her previous condition anyway at this point.
It's hard to look at her today and imagine just how much constant pain and discomfort she must have been in. We believe (Pediatrician included) that she is about 2-3 months developmentally delayed (primarily due to the pain), but that she is now progressing so rapidly that she should be back on track by 12-18 months. She will be 10 months old next week.
Day 34 (week 5)
Didn't expect to be adding to this so soon, but... Alright! Erin's crawling! She had a great day today and was very physical (rolling, pulling herself up to standing, zooming in her walker). All of the sudden at about 8:00pm, I was sitting on one side of the room with a glass of pop (she loves glasses and cups), and she looks over, smiles and starts crawling forward. It took her a few minutes to make it, and she stopped a few times (and had a slight sidewinder motion), but she did it all by herself. She continued crawling around for about twenty minutes.
Guess it's time to start baby-proofing ;-)
Day 180 (week 26 - January, 2000)
Finally - a new update (albeit short)! Erin just had her 1st scheduled post-op MRI. We have a meeting with Dr. Crone in a few days to review the complete results and see the films. Dr. Crone was nice enough to not make us wait in complete uncertainty and called us the day after the MRI. He said it appears that the tonsils have as yet not shrunken or moved back upward - but all pressure seems to be removed and Erin has very good CSF flow. And there is no evidence of any syringomyelia or hydrocephalus. This alone is good news to us because it was not the displacement per se that was the problem, but rather the pressure on the brain. More details after our appointment.
Erin is now 16 months old. Her general condition is very good except for the fact that she still does not sleep through the night. But she only gets up once, from about 2-3 am, and otherwise sleeps from 9 pm to 6:30 am consistently. She has no apparent symptoms of head pain or eating disorders. She does however appear to be several months behind in overall motor development - but she is progressing constantly. She has been walking for about one month (still with some balance problems) and she still does not talk, except for an occasional "Dadda". One area of concern for us is that she does seem to get fatigued quite easily and frequently lays down on the floor to rest for a minute or two.
In other aspects of her development, it's been a rough December and January - but in a "normal" way. She has had a few more ear infections and a virus, and she is teething like there's no tomorrow - four molars coming in at the same time right now, so she is in some discomfort. But she eats well and plays hard, and best of all has a completely "new" disposition.
Day 194 (week 28 - February, 2000)
Well, this is probably the end of the updates for quite some time to come. Erin was given a clean bill of health today from our neurosurgeon, Dr.Crone. He believes the remnant "conditions" we described at day 180 are normal behavioral issues, not physical ones related to the Chiari.
We're not out of the woods yet, however. Because Erin's skull is still developing, there's a possibility that her skull may grow back and cause another problem. For that reason she will get another MRI at her 2nd birthday, and then yearly MRIs until at least age five. If she develops any recurring symptoms then she would go in sooner. If there were any problems then she may require another round of surgery.
However, her recovery has been remarkable and we're definitely enjoying the "new her"! We'll just have to face whatever comes up whenever it does.
It has been great having the support of everyone who has taken the time to read this and respond to us. And it has been a great venue for documenting Erin's case. I'm happy to report that also today this page has exceeded its 1000th hit since we posted it 6 months ago. Thanks to everyone has has visited - we hope this information has been helpful, or at least insightful.
Here's a picture of Erin today - 7 months post surgery (and none too happy about getting her picture taken!)
URGENT UPDATE - March, 2000
As cruelly ironic as it is, only 4 weeks after Erin was given a clean bill of health from her Chiari she has now been diagnosed with Acute Lymphoblastic Leukemia (ALL). What can we say - we are devastated.
We all get to go through this entire journey again - only this time in the realm of childhood cancer.
Please find it in your hearts to pray for our Erin in the long battle she now has ahead of her. Thank You.
NOTE: All reports will now include both a Chiari and a Leukemia report. We can't guarantee there will always be information on both conditions in every update, so please bear with us. Thanks.
As far as we can tell, Erin currently has no lasting problems related to her Chiari except that we believe she is approximately 4-6 months developmentally delayed. The doctors don't believe she has a permanent disability, it's just that for most of her first 12 months she was in extreme pain and/or discomfort so she never had a chance to be cognitive. She is scheduled for physical, occupational and speech evaluations in June. We expect she will need several forms of therapy.
The days go on.
We won't be as clinical about her Leukemia treatments as that could takes pages - and honestly, we're just tired of clinical-speak right now.
As for the Leukemia, here are some details. Acute Lymphoblastic Leukemia (ALL) today has a "cure" rate of about 80%. That's drastically different from the 0% of 1970. But as one physician put it "don't listen to statistics - your rate will be either 0% or 100%. There's no 80% cure." So we try to go through believing it will be 100%, but obviously any setback brings to mind what the only other alternative is.
The first month of treatment was difficult. A heavy regimen of chemotherapy and steroids. The goal is to get into full initial remission within four weeks. That's why the chemo is very heavy. If that happens, you're on the good path. If it doesn't, well... we don't know. Our first disappointment with Erin came at two weeks. The doctors had told us that 90% of all children who show in remission at their four week point, also showed to be in remission at two weeks. So we were hoping... Well, Erin wasn't in remission at two weeks, so we had two more weeks to be fearful of the worst. At exactly four weeks Erin was in remission.
Don't get your hopes too high. The chemotherapy treatment for Leukemia lasts about 2 and a half years. So while her four week milestone is a great achievement, there's a long way to go yet. You have to be off treatment for 2 years before you're considered "cured". So in reality, best case, that's four to five years after you were diagnosed. That's a long haul.
Erin also went from the petite 18 pound girl you see in the picture at the top of this page to a very large and round 25 pound girl in three weeks thanks to the steroids. Needless to say she was miserable and couldn't move very well. Remember, that's about a 39% weight gain in three weeks. For some of us adults, that's like gaining 80 pounds in three weeks.
Well, we're currently finishing month #2. Erin has been sick and they took her off her chemo treatment for two weeks to try to get her immune counts back up - she currently has very little immune system (ANC counts under 200). She's back on steroids to try to bring her counts back up (steroids do that). But boy do those steroids make you cranky. She's acting fine but we're on germ patrol this week to make sure she doesn't catch anything.
As for us, well, we're drained as you might imagine. It's just too much to breeze through easily. And unfortunately the rest of life doesn't stand still for you while you deal with this. There are still jobs to go to, bills to be paid, a house to take care of, and a wonderful 4 year old son who we don't want to miss growing up.
that's it for now.
July 5, 2000
Erin had her evaluations in June. She just turned 21 months old. Her evaluations showed her current development levels to be at about 10-12 months in gross motor skills, 14-20 months in fine motor skills, and only 9-10 months in speech. Her fine motor skills are great (hey, we take what good things we can get!). Part of her problem with gross motor skills may be related to imbalance from the Chiari, or simply from delayed development after the correction. In addition, the chemotherapy also causes deterioration of muscle and muscle control which amplifies the problem.
As for speech... well, Erin still doesn't say anything except "yat" which is her sound for Yes. Everything else is just baby babble like "babababab" and "bbblleeeeee". I think Lisa will have a nervous breakdown the day Erin says "mommy". She's waited almost two years to hear something.
Erin is now currently undergoing physical therapy and speech therapy.
Chemo - round 2
Erin begins her heavy second round of chemotherapy today. This round will last about two months and she'll be pretty sick off and on.
And unfortunately, this will be the end of her hair. She had the longest black hair when she was born - you've never seen a baby born with longer hair.
This is a depressing stage for Lisa. But hey, Erin looks pretty in hats too.
July 7, 2000
Sorry to keep posting bad news...
Note: this entry was rewritten 7/21 due to the file being inadvertently deleted.
Unfortunately we have a new and critical problem. During a routine spinal tap (for her Leukemia) last week, it was discovered that Erin's spinal fluid protein level was completely out of control. Normal values are 25-40 with "elevated" values being 90-100. Erin's count was 3400. Yes, that's not a typo - three thousand four hundred when it should be forty. Her count can be slightly elevated by her Chemotherapy and could be slightly elevated if her CSF flow was again obstructed by her Chiari - but both Hemo and Neuro said those elevations would be small.
Our neurosurgeon was very straightforward. This signifies that Erin's brain is deteriorating (i.e. dying), but they have no idea why. Today she is undergoing an emergency MRI and they are planning a ton of neurological tests. But basically they are at a loss as to what is happening. She has been off of chemo for two months prior to this (yes she did have some sort of viral infection during that time which is why she was off chemo) - so no one suspects the chemo drugs.
As you can imagine, we are dazed, confused and beside ourselves. We're trying to maintain a calm perspective, but the words "her brain is deteriorating" are a difficult obstacle to get around. If anyone out there has ever encountered information regarding extremely high spinal fluid protein levels please email us at: email@example.com. Thanks so much.
We'll post results and further developments as we know them.
As we expected, Erin is starting to get nauseated from the chemotherapy and losing her appetite. She is also beginning to get very "clingy" with Lisa and screams pretty much any time Lisa puts her down. Erin is Lisa's new sleeping partner too - daddy is now in the spare bedroom. At least we get some sleep in this arrangement.
July 11, 2000
MRI Results and Correction
We were told today that Erin's spinal fluid protein value was 1374 not 3400. So it's 35 times higher than normal instead of 85 times higher. The implication however is the same.
Erin had her MRI yesterday. The preliminary reading (by the radiologist and the Neuro nurse) was that there was plenty of CSF flow (yeah for the Chiari!), and there was no sign of blockage, hemorrhage, tumor, cancer, brain shrinkage, etc. that they could see in the scan. The only item was a slight increase in white matter which is "normal" for active chemotherapy patients. Unfortunately, we don't know if this is good news or not. Obviously you're happy when you don't find anything - but half the battle is finding out what's going on.
The neurosurgery and neurology teams met today to discuss the results in detail and we are supposed to meet with them soon - not soon enough. At that point we expect to learn what tests are planned next and then we meet with Hemo again for another spinal tap to draw fluid for the tests.
One final note. Thanks to everyone who has sent wishes, prayers, thoughts and information. It means more than you know.
The nausea, unhappiness, clinginess and sleeplessness continue. She's sooooo tired - why won't she ever sleep?
July 16, 2000
Our church had a special service today (Sunday) just for Erin. The service was specifically to "ask for a miracle". It was actually a very sad service and has made us think about losing Erin. A lot of crying today...
July 19, 2000
No news yet.
Erin was admitted to the hospital today and taken off chemo temporarily. She has been sick and running a 101 degree (F) fever for the last day. Her ANC count has dropped to 10 (normal is above 1000) so she is considered immune depressed now. We're not sure what she has, but she'll stay in the hospital (and mommy gets to stay too!) until her counts come back up and her fever is gone.
This is a "normal" occurrence during this stage of her Leukemia treatment, and we were forewarned about it. So we're prepared for this one. Still no word from the Neurology team. We expect to hear something today or tomorrow. Erin was scheduled for a spinal tap Friday. Hopefully she'll be healthy enough by then to get it.
July 21, 2000
Miracle or Mistake?
Call it what you like, but Erin's results from her spinal tap came back "normal." Her protein level was back to 16. A far cry from the "1374 - her brain is dying" number. The hospital record official states that the reading was a "fluke" and there is no explanation for either the high reading or the recovery. They have cautiously said that they still don't believe it was a lab error. So... thanks again for the many prayers - they have saved her life.
Still in the hospital
Erin's fever has come down and her counts are going back up - but slowly. Her ANC needs to be above 250 before they will release her. She's at 76 now. Probably another 3 days till she can come home.
July 24, 2000
Erin came home from the hospital today. She is extremely fatigued and very, VERY clingy with Lisa.
July 30, 2000
And back to the hospital we go
It figures that our first night out together in 6 weeks without the kids we come home to find Erin with a 102 degree fever. She was admitted to the hospital at about midnight with a 103 degree fever and her ANC count was back down to 30. So much for having a night out.
Plus, we woke our neighbors up to drive us to the hospital (we had both had a few drinks and didn't think the 30 minute drive would be too safe with us at the wheel) and they unfortunately ran into the fire hydrant at the end of our driveway when pulling the car out. And we just got it back a week ago from the shop - having done the same thing ourselves! You just have to laugh...
August 5, 2000
Still in the hospital
So, it's been 8 days in the hospital so far. Erin's ANC counts have been all over the place due to the steroids. They were up to 3000 but are steadily falling, so we're not sure if they'll release her soon or not. She has been off of chemo for several days now.
Her biggest problem has been an infection in her rectum which is making her life pretty painful right now. You definitely know when she's had a bowel movement - you can hear her screaming in Alaska. But she is getting better. Today she spent most of the day happy and pain free. Not much appetite, but she has more strength than a week ago. That's it for now.
August 20, 2000
The calm before the storm...
Sorry, forgot to update the site when Erin got out of the hospital. She was in for 11 days, and has now been home for two weeks. She was supposed to start her delayed intensification phase last week, but her ANC was at 950 and they want it at 1000 before starting - so she goes in tomorrow. The chemo is very heavy for about 3 weeks. This is supposed to be the worst stage of the whole thing - meaning the strongest chemo, so Erin will be very sick.
What a wonderful reprieve the last two weeks have been though. Erin has been happy, and is doing well in therapy - AND she just started saying "mommy"! Okay, she says it for everything, but it's finally a start. It's been so much fun with her these past two weeks that it's going to be hard to see her so miserable.
Here's a picture of Erin today - bald but happy!
Sep 10, 2000
And still waiting...
Erin still has not started her delayed intensification phase yet, her ANC has been hovering between 650 and 950 now for almost 5 weeks, but she has not crossed the 1000 mark - which she needs before starting. They did a marrow pull last week to be sure the Leukemia was not active again and everything appeared fine. So... we wait. All indications point to a viral infection of some sort that she just can't get rid of. Hopefully now next week her counts will be up. If not, they'll do another marrow pull. Of all things Erin goes through, the marrow puls are the most painful - it's hard to watch.
Well, the reprieve continues. Erin has been happy, and is still doing well in therapy. But she hasn't been sleeping through the night again. She's up two or three times a night, so we're all a bit sleep deprived and cranky. But she's just being the happiest little kid.
Here's another picture of Erin - a big girl now, eating at the table!
Erin's birthday (#2) is coming up fast. And it's a fitting coincidence that our local "Light the Night" fundraising walk for the Leukemia & Lymphoma Society is happening on her birthday. If anyone wants to donate to the Leukemia Society in Erin's name, please contact us at firstname.lastname@example.org. Thank You. That's it for now.
Sep 28, 2000
After 7 weeks of tension (last week they told us Erin may be relapsing and to prepare for that possibility) Erin's ANC counts miraculously went up to where they needed to be (1300) and her blood and marrow were clean - so she started her delayed intensification chemo phase. All we can say is - Hooray! A week ago it looked like we would be headed for high-risk area, and now, all is back on track. It's probably because we finally just said "screw it - if she's relapsing at least let's let her have fun." And we let her play outside and with other kids for the whole week - just like she wasn't supposed to. And lo and behold, NOW her counts come up. She really is out to give us a nervous breakdown!
Also, last week Erin celebrated her 2nd birthday! The night of her birthday was the Light the Night Walk for Leukemia sponsored by the Leukemia and Lymphoma Society. We set out with a goal to raise $2500 for the Walk. A pretty lofty goal we thought.
We ended up with over $8700! It was a wonderful experience. Here's a picture of Erin and her Oncologist ("Dr. Cindy") during the Walk.
UPDATE: Problem #3 - Dec 18, 2000
Is three the Lucky Number??
Sorry we haven't updated the Site in almost 3 months, but the last few months we've had to come to some new realizations and come to terms with some new knowledge. By October, Erin had gone through about four months of physical and speech therapy, but was still very delayed. Even her therapists started to believe that her delays were from more than the side effects of the Chiari and the chemotherapy. We took Erin to a Developmental Pediatrician. When she was there, one of the other doctors in the Developmental unit stopped by and commented "She looks like a William's baby." So she did, commented the other doctor and we scheduled the genetic testing. Two weeks later, Erin was conclusively diagnosed with William's Syndrome.
After all this time, we finally had the answer we've been searching for - for 2 years. Erin has a rare genetic disorder which is the malformation of the 7th chromosome - William's Syndrome. Put bluntly, William's Syndrome indicates mental retardation and also has physical effects on the heart and kidneys. She has now been through a battery of tests, and at this point her heart is normal. This is a very good sign. Unfortunately, her delays are here to stay. Like all genetic disorders (and like Chiari for that matter), Erin has many of the symptoms associated with William's, but also is missing several. The most notable being that Erin doesn't seem to be bothered by loud noises. Most William's kids are.
Well, this is the third issue for us with Erin, and this one has been particularly hard for us. It's not that we're trying to be selfish, but one of the biggest sources of hope during the Leukemia has been that if she can make it through this then we'd get to share a lifetime with a beautiful normal little girl - albeit one with a big Chiari scar. That picture of "normal" has now changed dramatically. Lisa's "mom" dreams, selfish or not, of seeing her daughter get married and having grandchildren may never come true. William's can be a moderate to severe handicap. Erin may need long term care, and we need to plan for her future as well as our own. Just a new set of challenges and rules - but one we weren't quite ready for since we were already in crisis mode over her Leukemia.
I think now that the dust has settled, everything is fine. We'll just never know what each day will bring with Erin - so we need to live each day as it comes. She will always be our special little girl, and I think she'll end up surprising everyone. Heck, why should she stop now!
On a good note, we got a chance to take the kids to Disney World last week! I was speaking at a conference and everyone came along. We think Erin had a good time, although she had a sinus infection which left her a bit cranky at times. But what would any vacation be without a sick child?!
Here's a photo of Erin having fun with her big brother - who by the way is the best big brother any little sister could ever have.
On a related note, we've been told that doctors are seeing a link between William's Syndrome and Chiari. Basically, a high rate of children with William's Syndrome also have Chiari. Stay tuned.
Erin begins her second wave of Delayed Intensification in a few weeks. She'll get really sick and be hospitalized - so we're getting prepared. Right now she's off of chemo because she's been having a hard time fighting her sinus infection.
Dec 24-25, 2000
Ho Ho Ho!!!
Well... whenever you wish something doesn't happen it always does. Erin unfortunately spent Christmas Eve and Christmas Day in the hospital, and she's still there (today is the 26th). She's been fighting some kind of virus for over a month and on Saturday started getting pretty cranky (two days after the last antibiotic was finished). By Sunday (the 24th) she was pulling hard on her right ear and screaming. So off to the hospital we went. No fever yet but the CBC they drew revealed that her ANC was at 0 (Zero). After sitting in the little emergency room (8x8) for four hours I lost my cool and Erin finally started a fever. She was admitted right away - I don't know if it was the fever or my attitude that finally got the ball rolling.
Lisa spent the day at church and with family and then she relieved me and spent the night as they gave Erin IV antibiotics. On Christmas Day, Erin's counts were still at zero. But the hospital gave her a 6 hour "pass" to come home and celebrate Christmas between her antibiotic rounds. After that it was back to the hospital for both Erin and Lisa. All indications are that she'll be there for several more days - her counts need to be up and climbing before she'll be released.
Well, it's late - and that's all we know for now. It's a fitting tribute to the year we've had!
Jan 17, 2001
Happy New Year!
Nothing new. We're now about 18 months post-op. Erin continues to show no signs of having any Chiari symptoms returning. This is important because we were cautioned when she had surgery that her skull may continue to form and recreate the problem - causing her to need a second surgery. To date this does not appear to be a problem.
Today is a terribly sad day...
Yesterday our good friends from the Clinic suffered all of our worst nightmare. Tyler, their seven year old son who has spent a lot of time with Erin in the Clinic, died. Tyler was at home with his family for his last two weeks. Tyler was a wonderful, cute little boy who was full of life. Three months ago Tyler was on the road to recovery from his bone marrow transplant. In December he relapsed and he and his entire family spent Christmas in the hospital along with Erin. As every parent of a terminally ill child can tell you, there are no words of wisdom or consolation that can make this right. Our thoughts and prayers are with Tyler's family as they begin their longest journey - the journey of having to live the rest of their lives without the company of their beautiful son and brother.
As for Erin, she is not doing well this time with the delayed intensification. The chemo has completley wiped her out. She vomits constantly, won't eat or drink (her mouth and throat are filled with sores), has lost considerable weight, and sleeps constantly - as long as someone is holding her that is.
Hopefully for us, the worst of this will be over in about four more weeks. We expect that she will probably be admitted into the hospital within the next week - she's going to need blood and fluids soon.
Other than that, we had a wonderful belated Christmas and New Year. Thankfully we had those good, fun days before this round started. At right is a picture of Erin in her Christmas dress. The funniest thing about it was that she knew she was cute in her dress, and played the part! That's all for now.
Feb 26, 2001
This is a very brief update until we have some more time, I'm sure you understand...
Nothing new with Erin's condition. However, one note on our favorite front (of which we never speak): insurance. We were recently informed by our insurance company that in their medical opinion (oxymoron?) Erin was recovered from her condition(s) and no longer qualified for therapies. Ha, ha, ha..... well, I guess it would be funny if they weren't serious. Obviously the battle on this one is just beginning. We'll provide updates on this as they come.
Erin has had a very difficult time the last 5 weeks with this round of chemotherapy. She has been hospitalized twice (she is currently there) for dehydration and viruses. She had a feeding tube in place in January because her mouth and throat were filled with sores for two weeks and she wouldn't eat or drink anything for about 10 days. She lost five of her 23 pounds. Currently the chemo has made her urine and feces extremely acidic so her bottom is red with open sores. The virus has also given her diarrhea which makes the problem worse. Lisa and Erin will probably be in the hospital for another four days.
Erin didn't have nearly this amount of problems with her first round of delayed intensification so no one knows why she's having such a hard time now. While this can be a time of stress and strain, as your "normal" world gets put on hold, everyone here is holding up surprisingly well
July 31, 2001
Okay - we've been negligent and haven't updated the site in a while, sorry...
Well, Spring has blurred into Summer and Erin has been doing so well that we had almost forgotten she was sick. Okay, not really, but she has been doing very well, and she's been so happy it is wonderful to see her being a kid. Here's the skinny...
Erin will be due for her two year follow-up MRI in the next few months. However, she seems to be doing very well. Most of her significant problems attributed to the Chiari have not returned, as far as we can tell. She doesn't grab her head and scream spontaneously, she doesn't gag or throw-up, and no sleep apnea to speak of. Her balance however is still very shaky, and she is behind in both fine and gross motor skills. She has physical and occupational therapy weekly to try to develop these skills. While she doesn't appear to have many of the severe Chiari symptoms returning, the big test for the MRI is to see if she still has good fluid flow to the brain. Hopefully that will be the case.
Erin is now several months into her final 18 month maintenance phase. With good luck this will be the end of her active chemotherapy and in 3-1/2 years can be considered "cured". While she has appeared healthy over the past three months, her blood counts (ANC) have been very low signifying a problem metabolising her chemo. She's been off-again/on-again while we try to find the right dosage. Right now she's on 1/4 dose. The issue here is making sure she stays on chemo and doesn't have keep stopping for weeks on end - which feels like playing Russian roulette.
Erin is also still heavily speech delayed. This is in large part due to her hospitalizations earlier this year because of complications from her chemotherapy treatments. Her speech therapist believes she is progressing, but still has not rebounded to where she was in January before her setbacks. Her sounds are not as clearly defined as they were then.
All in all though, Erin is doing very well. She's in great spirits, her hair is growing back quickly, and she has been fairly healthy. We can only keep hoping...
New: William's Syndrome Update
Erin will be three in September. She still can't really talk (although she has roughly a 20 "word" vocabulary that she will use when she wants to - but that has degraded with her Leukemia setbacks), she is a bit unbalanced, she has a hard time sleeping through the night, throws some great temper tantrums, and has absolutely no desire to start potty training. Cognitively she seems to understand a lot of what we say, because she reacts to the words properly, but she does not really get cause-and-effect relationships. Like "if you do A then you can have "B". She only hears the two distinct statements: "Do A" and "Have B", but can't put them together. She will start in a special mixed Preschool class in September.
|This past weekend we attended a regional picnic for the William's Syndrome Association. There were about twenty families there with children of all ages. Not only did the kids get to meet other new "friends" but it was a great experience for us as parents to talk with other parents at various stages of their child's journey through life with William's. Of course, all of our other kids had a blast too!
Not to disappoint you - here's a photo of the little Ham, playing beach bunny for the camera.
That's it for now - thanks for caring.
November 13, 2001
Boy, and I thought we were negligent before - it's now been 3 1/2 months since our last update.
William's Syndrome Update
Tonight on PBS's Scientific American Frontiers was a wonderful special on Williams Syndrome, hosted by Alan Alda. The program was titled "Growing Up Different". For the complete program and information, see the PBS website at:
What can we say, we've had a pretty nice autumn except for those usual things like therapies and insurance issues. But all in all it's been nice.
Erin started pre-school and is in a mixed class - several handicapped and several "normal" children. She loves it, and she has made progress. Since there's really nothing new medically to tell, I thought I'd just show some pictures below of the last few months. Hope you don't mind!
|Cosmo, here I come!
|At the Light the Night
|On Daytona Beach,
|At the Wish Kids
|First Day of School!
|Happy 3rd Birthday!
March 18, 2002
The updates are slower, but that's good news...
Where did Winter go? It's strange, we've had no snow this year (well, none that you could actually call snow) and it's been uncommonly warm. Erin has been doing very well, except for a nagging ear infection that has gone on now for almost four months. She's been on antibiotics almost that long.
But the big news is... Erin's got a new little brother. He was born Feb 19th, and as you can see - Erin wants desperately to play "mommy". She tells us every time he cries (even though we're right there) and every minute is a "hold baby!" moment. Obviously we've all been a little busy right now, but here is the rest of the update:
Erin's two year follow-up MRI came back with all results being positive. We're very grateful because there has always been the potential that this condition may reappear and that she may require another surgery. So, we can hopefully breathe easier on this for yet another year.
Erin's blood counts (ANC) have still been fluctuating the past few months and we've finally set on her "correct" chemo dosage. For Erin it appears that her body can only metabolize what would normally be 1/4 of the standard dose. So that's where she's staying for the remainder of her maintenance phase.
Erin remains heavily speech delayed - but her vocabulary has increased. She at least tries to verbalize more, even though we can't always make out what she's trying to say. Erin has definitely hit the "Why?" and "Huh?" 3-year old phases. These are her two favorite words now (along with "Baby crying").
If all goes well, Erin will complete her chemotherapy in May. We then begin the long two and half year wait - hoping that she doesn't relapse, and can be considered cured. While we would love to celebrate, this is still not the time...in fact, this will probably be the most scary time to date.
On a good/fun note, Erin has become associated with two wonderful organizations:
- Adventures for Wish Kids - a great organization that provides fun family events for families of children with life threatening illnesses. We have had the good fortune to attend a Halloween Pumpkin fest (complete with hay rides) and a Christmas party (hosted at Paramount's Kings Island). You can learn more about this volunteer organization by visiting the Adventures for Wish Kids website.
- Team in Training - a wonderful group of people associated with the Leukemia and Lymphoma Society. These dedicated athletes compete in various endurance races (such as marathons) across the Country to raise money and awareness for Leukemia research. This year, Erin has ten runners/walkers who are training and competing in her name. You can learn more about these champions by visiting the Team in Training website.
William's Syndrome Update
As noted before, Erin's vocabulary has improved but her clarity of speech has a long way to go. She primarily still uses incomplete sentences and one or two word phrases. Her balance has improved somewhat and she can now jump with two feet (about an inch off the floor) - a huge milestone. Still no potty training, but lots of attitude. Cognitively, over the past six months, she does not seem to have significantly improved in her understanding of cause-and-effect relationships. This is still a big problem area. She has started in a special mixed Preschool class, which she loves. Oddly enough, everyone in the school knows Erin, and every teacher says Erin is "hers"! She's just too cute...
One other big note: the 2002 National Convention of the Williams Syndrome Association is being held in July in Long Beach, California. You can get more information at the Williams Syndrome Association website.
That's all for now. Have a Happy Easter!...
May 5, 2002
It's time to start celebrating a few good milestones...you have to take them when they come!
Erin remains (apparently) symptom free from her Chiari - we couldn't have hoped for more.
Now for some good news!
Wednesday, May 1st was Erin's last clinic day! No more trips to clinic for 4-hour chemo and anti-biotic drips, no more spinal taps (well, none for a while). Now she just gets monthly checks for the next 2 and 1/2 years. At which point, if all goes well and we don't relapse before that, she will be considered cured. Now, in all honesty, this is the beginning of another bad stage for us as parents because it's the "wait and see" stage. And 2.5 years is a long time to wonder and worry about everything. But we'll take it over the Hell she's been through for the past 2 years. Here's keeping our fingers crossed.
On Thursday, May 2nd Erin had her central line (c-line) removed. It was so weird to see her without it. She's had the line and had to wear her protective netting for over two years. So far we've still sat down each night to scrub her line - only to find it gone! She'll now get to do all those things she hasn't been able to - like take a long bath in more than an inch of water, go swimming without an immediate change of her dressing and cap (end of the line), play in the dirt and mud like any other 3 year old. This is truly something to be happy about.
We did make the mistake however, of making a big production of it the night before her line came out. Erin proceeded to grab her line at bedtime and say "Line out now?!" - as we're both screaming "No!!" So that was almost a bloody emergency. She finally understood that the doctors had to take it out and she shouldn't. That was a less than restful evening as we wondered how we'd find her in the morning.
One more note: Erin has only 10 more days of in-home chemo (her pills) and then she is done with that too! It's almost anti-climactic. Things are just coming to an end with no fanfare. But these are good things - it's just scary because you feel like you're giving up the lifeline you've had for so long...
William's Syndrome Update
Erin is starting to show her affinity for music. Even though she is still significantly speech delayed, she is starting to sing songs frequently. While she doesn't say every word correctly, she does know each verse of songs like "Twinkle Twinkle" and says a majority of the syllables. And every time she hears music she wants to bounce and dance.
Erin was just evaluated for Music Therapy and really responded well to tasks when directed through music. So, now it's off to try to get this covered in our IEP at the end of May. Apparently this is a battle with school districts nationwide even though it is covered under the Individuals with Disabilities Education Act (IDEA) signed into law in 1997. Ahhh... what would we do with our time if we didn't have these battles to fight all the time!
That's all for now. We'll try to have some new photos and updates soon.
July 30, 2002
Parting is such sweet sorrow...
Today Erin's best friend and our good neighbors, moved away to South Carolina. It's especially hard because Erin doesn't understand the concept of moving away forever. And Harrison was truly her best friend and playmate. It's definitely going to be hard to adjust.
No new news.
Three months into the "no-chemo" period and Erin is doing well. Her counts have been great and she's been very healthy - and especially happy. She gets checked again this week, so we're keeping our fingers crossed.
William's Syndrome Update
We attended the WSA (Williams Syndrome Association) convention in Long Beach along with about 350 other families. Great conference and we picked up a lot of good information.
We're now 4 weeks into our Music Therapy battle with the school district. Hopefully something will be resolved soon - this could be a long discussion, but I won't go into it now.
That's all for now (short update). Thanks for reading!
August 5, 2002
William's Syndrome Update
Today we learned, to our sadness, that Trent Johnson passed away after suffering heart failure several days ago. Trent was a wonderful little boy, 18 months old, who had overcome three open heart surgeries in his short time here. Many children with Williams Syndrome suffer severe heart problems.
Our thoughts are with Trent's family. Trent's mother was a huge part of our internet support group - providing hope and comfort to many families. Trent's strength and courage will not be forgotten by any of us.
January 17, 2003
Wow, has it really been almost 6 months since we've updated the site?....Sorry, now I know why everyone is asking about Erin!
Erin had her annual MRI in November and has been cleared for another year. This is good news. As far as we can tell, Chiari is no longer an issue. Let's keep our fingers crossed.
Erin has been off chemo for almost 8 months now and is still in remission! The next 4-6 months are a critical time however. Apparently, most of the children who do relapse after going off chemo do it in this period. Erin had a clinic visit last week and her counts were great. We're happy - but guarded.
Over the past four months the only real reminders of Leukemia we've had have been the events for Team in Training and Adventures for Wish Kids that we've attended. We again thank these organizations for everything they do.
Erin will probably take her Make-A-Wish this year and she's starting to talk about it now. She wants to go to Disney World and we just rented a video that she's watched over and over and over and over and....
Here is an updated picture of the princess (now age 4):
Isn't she cute!
William's Syndrome Update
Physical: Erin's therapies have paid off in that she is much more stable than she used to be. Also, she just saw her cardiologist and has been given a five-year clean bill of health. This is excellent for a child with Williams Syndrome. We are very lucky that Erin's heart is as healthy as it is.
Education: As we eluded to earlier (in May), we have had to hire an attorney to try to get the school district to understand Williams Syndrome and the fact that there may be unique ways she need to learn, including using Music Therapy. My God it is an amazing battle - but we won't bore you with details. After seven months and thousands of dollars of legal bills, the school has finally agreed to an outside evaluation of Erin. Yippee - but we should have been there six months and thousands of dollars ago...
As an example of understanding the obvious, the school told us that learning her phone number was neither age appropriate for Erin, nor within her capabilities for this year and they would not include that as a learning objective for her in 2003. But, in just 3 months of Music therapy (1 hr per week which we paid for ourselves) Erin can recite not only her phone number on demand, but also spell her first and last names. I'd say there's something to that Music Therapy connection, wouldn't you?
Well, that's it for now. More updates to come later.
December 5, 2003
Well, another 11 months has flown by and here I go being neglectful again! Oh well - suffice it to say, Erin is doing great so we've all been enjoying being a family. Here are some updates.
Erin continues to show no signs of her Chiari returning. She has scared us several times by cracking her head on something or whipping her neck around - but she's proving to be a tough little kid!
Erin has been off chemo for 18 months now and is still in remission! She is supposedly past one of the critical times and she is lookling good. Erin had a clinic visit last week and her counts were fine (a little down from a virus she had, but nothing concerning). We're happy - but as always: guarded.
Erin and Lisa continue to support Team in Training, and this week is the wonderful Adventures for Wish Kids Christmas party at Kings Island Amusement Park. Bound to be a lot of fun. We again thank these organizations for everything they do.
And, it's now official: Erin's Make-A-Wish trip to DisneyWorld is confirmed!
William's Syndrome Update
Physical: Erin played soccer this year - how's that for physical?! Okay, she actually wandered around the field saying "Hi" to everyone on the other team - but that's as close to playing as she'll get for a while.
Here is a photo of her playing soccer.
Erin has been receiving hippotherapy (horseback riding) in addition to her PT, OT, Speech and Music Therapy for several months now. She's a very busy girl! Her balance and strength have improved immensely. She can actually walk (hand over hand) across a set of about 8 monkey bars with minor support (she can do 3 all by herself), and the girl has some biceps!
Here is a photo of the little equestrian:
Education: Erin turned 5 in September: Happy Birthday! She's doing well in her preschool and she is really starting to talk in better sentences. Still has a long road ahead, but we'll take all the progress we can get. Also, we never updated you on our legal issue with the school, so here is a brief update. The school performed their new evaluation, and to their credit it was a fairly extensive testing exercise - took several months. Based on the results of the testing, we have all come to a better understanding of how to work together. The school has changed some things, and is providing the services we've requested - and we are working with the teachers and therapists to support them, and we all compromised on the music therapy. The school is providing a music therapist for the entire class (not personally for Erin) so she gets the service, but the entire class benefits from the therapist's lesson as well. So far it is working out well. Hopefully we can build a relationship now with the school to help Erin in the years to come.
One thing we can't forget to mention is that Erin was the flower girl in the wedding of her Assistant Teacher in June. Erin was adorable - and was in awe of the entire thing. She did a wonderful job standing on the stage the entire wedding! Well, okay, she did walk right up in the middle of the vows and talk to the minister, but hey - what did you expect!
Here are my two favorite pictures of her at the wedding.
And lastly, here is an updated Holiday picture of the princess today (now age 5):
Well, that's it for now. Happy Holidays!
September 8, 2004
Well, another 9 months gone by.... Here are some updates.
Yesterday, Erin had her annual MRI to check her Chiari and see if there's been any change in the state of her elongation or CSF flow. Her scan came back showing no change over the last few years. So Erin has now been released from her Neurosurgeon FOREVER! No need for any more MRIs or checkups - unless of course she shows some symptoms taht would lead us to believe she was having a relapsing problem.
Obviously, this is great news and we're thrilled to feel like this one piece of the problem puzzle is behind us. Again, we give our warmest thanks to Dr. Kerry Crone whose careful surgery and care have helped Erin so much.
Erin has been off chemo for 18 months now and is still in remission! She is supposedly past one of the critical times and she is lookling good. Erin had a clinic visit last week and her counts were fine (a little down from a virus she had, but nothing concerning). We're happy - but as always: guarded.
Erin continues to stay cancer free during her checkups. Only nine months left till her 5 year milestone - not that we're counting (okay, we are!). Erin stays active with the Leukemia Society and Team in Training for their fundraising efforts. The least we can do...
William's Syndrome Update
Physical: Erin learned to ride a two wheeled bike (no training wheels) this summer - way ahead of schedule! And she's playing soccer again this year - and is doping much better than last year. Her physical strength continues to be a huge area of improvement and success.
Erin continues with her hippotherapy (horseback riding) in addition to her PT, OT, Speech and Music Therapy. Quite the schedule.
Education: Erin will turn 6 in 2 weeks! Erin just entered Kindergarten and while we had three IEP meetings to "work out" all the details, we finally feel like we have a good and cooperative team at the school (a new team). Her new teachers seem great, and everyone is starting to play well together! Our new school Principal has been excellent for facilitating a productive and helpful environment. He has no idea how much that has meant to us. Too bad Erin moves to a new school next year.
On a fun note, Erin toook her Make-A-Wish trip to Disney World in May and we stayed at the Give Kids the World village. It was a wonderful time for the entire family. You can see the full trip report at www.erinsmakeawish.com.
Well, that's it for now. Hope everyone had a great summer!
January 18, 2005
A quick update....
Well, this isn't an update on Erin as much as it is on Lisa.
In honor of 2005 being the year of Erin's *Cure Date* (hopefully), Lisa has taken it upon herself to join the Leukemia and Lymphoma Society's Team in Training group and run a Marathon in honor of Erin!
Obviously this is a huge commitment on Lisa's part - she has never run more than 5K before this year and never even considered she'd ever run a marthon. We're all very proud of her.
Of course, this is all about raising money for the Leukemia and Lymphoma Society - and Lisa is trying to raise $10,000 by April 1st, 2005. If you want to see details of this quest - or if you'd like to donate to the Leukemia and Lymphoma Society in Erin's honor, please see Lisa's marathon site at:
- www.racingforleukemia.com (no longer available)
That's it for now.
Note: This may be the last update for quite some time as there are no relevant medical issues to post.
UPDATE: June 30, 2010
A quick update....
The years they are a flying by...
Well, for all of you have asked what's happened with Erin: 5 years later and things are still going well. Of course Erin is now getting older and in 6th grade and hitting all those wonderful school/educational issues. But health-wise she is doing great.
Just got back from a very busy vacation in Orlando. What more can you ask than swimming with Dolphins and Disney!
That's it for now.
Note: Again, this may be the last update for quite some time.
UPDATE: June 3, 2017
Has it been 18 years since this journey began already...
Never doubt the power of hard work and high expectations...
Erin will be graduating from High School on June 3rd, 2017... and headed off to college...
Wow... what a life...
The following sites offer very good information and discussion about Chiari Malformation:
You can download my resource file which I collected over several months (via the above web sites and additional Chiari sites) by picking the link below. Please note that this is a 245 page Adobe Acrobat (pdf) file and is approximately 3MB in size so it will take several minutes to download. But it may save you a lot of time researching the sites.
The following sites offer very good information and discussion about Williams Syndrome: