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3coolkidsmom
March 17th, 2004, 01:42 PM
I think this subject has probably been addressed here before, but since I have not been able to post until now I wanted to add my two cents worth. I often hear parents of MPS children saying that their children were tested for hydrocephalus but found to be negative. They go on to explain that the only diagnostic tests that were performed were CTscans or MRIs. In our experience with our children, Ct scans and MRIs did not show any signs of hydrocephalus. They are used to determine ventricular enlargement. Because of storage material in the ventricular system, MPS children do not always have enlarged ventricles. Doctors also look for papilledema, but this symptom is usually the last to occur before blindness from hydrocephalus. It hopefully will not be seen in upon exam.

When my kids were diagnosed with communicating hydro, the only reliable symptom was increased inner cranial pressure, or high ICP. This can only be done through lumbar puncture. After our first child was diagnosed, we wised up enough to start insisting that lumbar punctures be performed during any surgery that our other two children were having. Having a lumbar puncture done under general anesthesia gives a more accurate measurement than one where the child is awake and crying. If your child is not having surgery and LPs are difficult, flouroscopy can make the procedure much less traumatic.

My first experience was with an nonbelieving neurosurgeon. My second was with Dr. Swift, and there is not enough space to write how great he is.

Barbara Pryor
March 17th, 2004, 03:06 PM
Amy,
I told everyone about the fabulous Dr. Fabio, as you called him at the conference in Texas. I really liked him especially when we talked with him and he was willing to let the pediatrician for Andrew call him and talk to him. He is wonderful, and not just pleasing to the eye. :D

Laura Brodie
March 17th, 2004, 04:06 PM
Oh wer'e not back on that one are we! :lol: love Laura(still jealous)

Sharon Cochenour
March 17th, 2004, 05:03 PM
Amy thanks for the input. I think I might see if Andrew's doctor will check during his next surgery. His ventricles are at the high end of normal anyway, and we could at least have a baseline.
Laura, you are too funny. :lol:
Sharon

March 18th, 2004, 08:59 AM
Amy, thanks for your two cents. I have been on this one ever since Chase had his shunt. You are so right. Communicating Hydrocephalus does not show on CT scans or MRI's but that is how they would check for typical children, to look for enlarged vetricals. That is why you need the doctor who is familiar with MPS kids. Chases venticals are very inlarged now even with a shunt. But I too would like to say this, the lumbar puncture was not accurate either in our case or my friends case Zach Jones,(11 yrs old hunters). In fact Zachs came out perfectly normal. It was only the CLINICAL signs the boys were showing that signaled high pressure. They were loss of speech, bladder control, holding head, crying, sleep disturbances, unable to put head on pillow,increased irratability, rubbing of the eyes.Also it really needs to be repeated often for all of the new members here with the younger children. The papilledema does not show either.This is by far a very important issue. Chase has had 5 VP shunts. and three brain hemmorages. and the doctor said had he not been shunted he would have died. He was 10 at the time and Dr. Goodrich in NY said he had to have suffered a long time prior to the surgery with Communicating Hydrocephalus. Parents need to really watch for this one. Thanks Amy and God Bless you and your family.Rosemarie

3coolkidsmom
March 18th, 2004, 10:47 AM
Rosemarie,
you are sooo right. Our kids are all quite verbal and able to tell us when thier head is hurting . When we were still seeing the nonbelieving neurosurgeon, Maddie cried all the time and would lay her head down at the table as she was eating saying her head hurt. What scared me into finding Dr. Swift was the day that Maddie asked me why it was so dark outside even though it was 3:00 in the afternoon on a cloudless summer day.

With Laynie, her headaches became more frequent and she became dizzy all the time. Sitting on our porch swing, riding in the car, sitting in the stands at a football game would make her so dizzy that she would throw up. Also, her headaches, which had been about a three on a pain scale of 1-10, went to an eight or nine and would last for hours.

With Spencer, we had been monitoring his pressure for 2 or 3 years. ERT seemed to help his headaches for quite a while, almost five years, but then his headaches returned and his next lumbar puncture showed an increase in ICP (600ml) He had also been exhibiting signs of mental confusion, repeating things and forgetting common words and names. (He does this to some degree anyways, but it became markedly worse) Spencer just had his shunt surgery a year ago, when he was 13.

In all three cases, VP shunting helped my children, although shunts carry with them their own set of complications as well. But VP shunts save MPS children's vision and lives.

Sheila George
March 18th, 2004, 11:21 AM
I have to ask a question here. I know that you all are talking about Hurler's and Hunter's kids, but what about Sanfilippo. Shelby has enlarged ventricals, but they tell me that this is not a problem. Shelby is none verbal and cannot express to me such things as headaches, dizziness, blurry vision, etc. All the information I read about Sanfilippo says that they do not need shunts, however I do know of 2 or 3 Sanfilippo kids who ended up needing shunts. So how do we know? Even at the conference in Ft. Worth, Dr. Swift and Dr. Muenzer said that Sanfilippo kids do not need shunts. Just wondered if anyone had any input on this?

We are going to the dentist in April and I am sure he is going to say she needs some dental work, which will have to be done under anesthesia, so you know what I am wondering, should she have a LP?

Paulina Jump
March 18th, 2004, 12:17 PM
I would like to know that also Sheila. Andrew's(MPSIII B) doctor wants to do a MRI. He is rubbing the back of his head and his eyes alot. He will cry and hold his head. About 2 months ago he was getting out of bed and would fall to the floor, turn white as a ghost and just lay there and would start to gag like was going to get sick. He was on the clonidine patch, they took him off and it seem to stop. They said it was probally lowering his blood pressure to much. His eyes are changing for the worst every 6 months. His eyes seem to be blue/black color at the corner of the eye lids. I asked he seemed to think it has something to do with the blood vessels.

Paulina

DebbieV76
March 18th, 2004, 06:58 PM
I'll add my name to the 'Sanfilippo-moms-who-want-to-know list.'
It's one of my greatest worries that Jon or Jason could be having pain--any kind of pain--and I wouldn't know if there was a major problem.

I always worry when Jon isn't his usual smiling self... headache, sore throat...broken leg? :? I don't mean to be silly... but it is a real concern...and probably my biggest frustration.

What are the symptoms here?

Thanks in advance.
Debbie

3coolkidsmom
March 19th, 2004, 01:37 AM
Debbie,

I have been thinking about what you wrote and I am in no way qualified to answer you with my kids' experience, however, I remembered reading something a few years ago that may help you.

One of my friends, Sue Rattman, who has a child named Adam with Hunter Syndrome, wrote a letter to courage in Sep. 19996 about Adam's experience with communicating hydrocephalus. Sue writes "all at once Adam's behavior, sleep pattern, and speech all took a drastic change for the worse. He woke up numerous times every night, repeated the same word over and over like a broken record, and was uncontrollable. Adam's personality underwent a drastic change. He was mean and constantly striking out at people." Sue continues by saying she made several phone calls but got nowhere and was told that she needed to face the fact that Adam was deteriorating due to MPS. After speaking to Dr. Muenzer who felt like Adam's symptoms were consistent with communicating hydro, Sue fought everyone in her path and Paul was admitted to Johns Hopkins for ICP monitoring, which revealed high pressure. Adamwas shunted at that time and Sue writes that he returned home his sweet natured self.

Sue closes with a note to keep searching until you find the answers and to remember that you, the MOM, are your child's best advocate. (I'm gonna put that on a resume someday!) Hope this helps a little, I can only imagine how hard life must be for the parents of nonverbal children and I'm so sorry...

3coolkidsmom
March 19th, 2004, 01:40 AM
Debbie,

I have been thinking about what you wrote and I am in no way qualified to answer you with my kids' experience, however, I remembered reading something a few years ago that may help you.

One of my friends, Sue Rattman, who has a child named Adam with Hunter Syndrome, wrote a letter to courage in Sep. 1996 about Adam's experience with communicating hydrocephalus. Sue writes "all at once Adam's behavior, sleep pattern, and speech all took a drastic change for the worse. He woke up numerous times every night, repeated the same word over and over like a broken record, and was uncontrollable. Adam's personality underwent a drastic change. He was mean and constantly striking out at people." Sue continues by saying she made several phone calls but got nowhere and was told that she needed to face the fact that Adam was deteriorating due to MPS. After speaking to Dr. Muenzer who felt like Adam's symptoms were consistent with communicating hydro, Sue fought everyone in her path and Paul was admitted to Johns Hopkins for ICP monitoring, which revealed high pressure. Adam was shunted at that time and Sue writes that he returned home his sweet natured self.

Sue closes with a note to keep searching until you find the answers and to remember that you, the MOM, are your child's best advocate. (I'm gonna put that on a resume someday!) Hope this helps a little, I can only imagine how hard life must be for the parents of nonverbal children and I'm so sorry...

Priscilla Raftery
March 19th, 2004, 10:40 AM
Thank you, Amy. Joey acts exactly as you described Adam Rattman from time to time and for days, weeks, or even months at a time. But Joey has Sanfilippo and like most of us, we've been told Sanfilippo kids do not need shunting or have the pressure. That is what Dr. Muenzer said at the LA conference in 2001. I'm not so sure now...what kind of doctor should we pursue this with, his neurologist? Geneticist? What test is done to determine pressure? Thanks for your help. Priscilla

MelI
March 19th, 2004, 03:29 PM
I am by no means knowledgeable on shunting and Sanfilippo BUT I know of a Sanfilippo child who was shunted and I know that his parents did ALOT of research on this. His name is Andrew Bowman and he had a cord blood transplant at Duke --- I think he is 3 years post-transplant. He has had multiple complications and in the past year had a shunt put in at Duke. His is adjustable which is different than the one that my son (Jake - Hurler's) had put in but nonetheless ... Andrew's website is http://www.caringbridge.org/page/andrewshope . They have also consulted a specialist in New York who is apparently very familar with shunting/MPS/Sanfilippo named Dr. Goodrich. If you go to Andrew's website and look at his 'Journal Hostory', they discuss it various times --- I found the info. on Tues. Aug. 12, 2003. I'm sure Ellen (Andrew's Mom) would be happy to share this info. with anyone and I believe her direct e-mail is on Andrew's website. I hope this helps a bit! :) I know how difficult looking for answers can be!

Take care, Melanie Irish
http://www.caringbridge.org/wa/jacobirish

March 19th, 2004, 09:34 PM
Hi, I too can only share what happened to my son and my friends son both with Hunters.Pat was treating Zach,(9 yr old ) with medication for his hyper activity and for sleep. My Chase was repeating words and phrases and had the above mentioned issues. He lost bladder control, could not put his head down to sleep, woke many times at night trying to get out of bed, was upset and throwing things, hitting my other children.He was hurting and unable to tell me.I knew it in my heart. I called Sue Rattman and asked for help. She shared with me her story. The doctors all said Chase had no communicating hydrocephalus. They said his brain was shrinking a dying. I begged and called and argued until I finally got to Dr. Goodrich in NY. He listoned and said my son had the clinical signs and agreed to take him into surgery saying,"If I open him up and find he has no high pressure than we have put him in for an uneeded surgery, if he has high pressure then we did the right thing and I'll shunt him" I said "Please do it" When Dr. came out he was a lttile shocked saying,"his pressure was the highest I have ever seen in a child, he would have died had we not shunted him" He said Chase must have felt like he had a Mac truck parked on his head.

But I also need to say that what we learned was this,MPS kids can have the total opposite of the typical symptoms in many areas than the symptoms that would normally present in a typical child and or another MPS child. For instance both Zach and Chase had low pressure vp shunts placed because both boys high extremely high pressures. (we both used a different set of doctors to gather our information from, I then brought her to Dr. Goodrich) Within 4 weeks after the surgery(they were shunted the same day by DR. Goodrich) Zach began to vommit and became lethargic. He was having a brain hemmorage. The same time Chase was out of control hyper, crying, fighting unable to sleep, would scream unmercifully when I brought him to the bedside. I called the doctor questioning the fact that Zach was having a problem maybe Chase too was hemmoraging. They said no Chase would be lethargic and vomiting just like Zach. He was not. I continued once a week to bring him to NY to ask for a scan to check, and was told no he would not be walking if he had a brain hemmorrage and they would not do the scan.. He had no typical signs of a brain hemmorage. I spent the nights driving him around in my car so my other children could sleep as he cried and cried holding on to the door handle. I prayed and begged God to help me . It was awful. It went on, the visits to NY and back, and the crying, the hyper activity and he could not stop moving. He could not stop walking in circles for hours.They thought maybe he was just adjusting to a lower pressure in his head as he was used to a higher pressure.Then they said he must have a behavioral problem related to Hunters.....NOT..... Finally after 5 visits to NY many phone calls, and 11 weeks, I called and said he was vomitting and lethargic. He was not..I lied. I should have done it earlier. They said get him in here now. They scanned him and said his brain had collapsed,he had a massive brain hemmorage and it was the size of half his brain. It had to have been going on for months they said. NEVER had they seen this before and Dr. Goodrich was flabbergasted as I cried to him saying "you didn't believe me"

When they took him to surgery the dr said he touched Chases brain and it was like a piece of clay, grey and stiff. It had been compressed with the pressue and the deposits, thus it was stiff and was not expanding as our brains would do. You see, the low pressure shunt was too low and it drained his brain. What happened after that was just horrible and this story is so long now.

I just wanted to say that our kids present in different ways than the typical which is what the doctors look for.It is up to us to be the advocate and question the doctors and get as many opinions as possible and relate other MPS stories to them.

Chase did not present the typical,and it was hard to get doctors to liston. Go with your gut instinct and pray alot, that is all I can say.It is hard.Call Dr. Goodrich if you need help.God Bless.

Rosemarie

3coolkidsmom
March 19th, 2004, 11:49 PM
There are two different ways to monitor intracranial pressure. The first, is by getting a baseline lumbar puncture, also called spinal tap. This can be done by a neurologist. Because the anatomical structure of our kids differs from other children, spinal landmarks can sometimes be difficult to feel. You can ask your neurologist if they think it would be easier to do the lumbar puncture under flouroscopy (using x-ray techniques to position the needle) and if so, a radiologist would do the procedure. Lumbar punctures can be repeated at intervals throughout the year to check for increases in pressure.

Another way to evaluate ICP is to temporarily implant an intracranial monitoring device (a small fiber-optics cable.) This requires hospitalization and anesthesia. A burr hole is made behind the hairline in the frontal region and the cable is inserted through the dura. The monitor is usually left in place for 24 hours or more. This allows ICP to be monitored when the child is awake, asleep, standing and sitting. This procedure must be done by a nuerosurgeon.

DebbieV76
March 20th, 2004, 06:32 PM
Rosemarie...I can't even imagine going through what you did with Chase. And it sounds like this is only part of the story. I'm so sorry.

I just hope and pray that the Lord gives me wisdom when I need it. When people ask how to pray for us now, that's usually what I ask for.

Thanks so much for everyone sharing here. It is really good to know the info will be here when I need it.

momtoty
March 22nd, 2004, 10:51 AM
Amy,
I too went through three "non-believing" neurosurgeons before finding Dr Swift. Ty is now 17 months old. He was dealing with the enlarged head, raised ICP via LP's, and as a young baby he was severly delayed. He could not hold his head up, push off his tummy, roll over, or sit at 8 and even 10 months old. We were told he only had low tone in his trunk and that he had hypertone in his extremeties. At 6 months a CT scan showed "benign external hydrocephalus" (I hate that term-there was nothing benign about his condition), he had ICP of 45 (or 450ml) at 7.5 months, but a MRI revealed "no change- still benign external Hydrocephalus" so still nothing. By the time Ty was 10 months he had bilateral papilledema, severe aggressive behavior, major sleep disturbance, and projectile vomiting (also banging his head into everything in sight)
After two more trys at neurosurgeons and several neurologists, Ty was now 13 months old and living in constant pain. After being told he could go blind from the papildema, by his neuroopthalmologist in Dallas (Dr Hogan- who is incredible) and that we needed to do ICP monitoring by indwelling device- I could find no one who would do it here in Houston. I got in touch with Dr Goodrich, who suggested Dr Swift. I called Dr Hogan, who got me right into Dr Swift, and finnally at 16 months, Ty was shunted ( no ICP monitoring was done- Dr swift did a LP himself- under sedation and Ty's pressure was 33(330ml)- we went directly to the shunt.
I can't tell you how much this has helped Ty! He is not in pain. He will actually lay his head on a pillow with a smile, and at his last eye exam with Dr Hogan- His optic nerves are now only elevated- no longer swollen.
What a great group of doctors you have in Dallas, they have me wondering why TCH in Houston gets all the kudos- they don't deserve it.

3coolkidsmom
March 22nd, 2004, 12:49 PM
Isn't it amazing what we go through!. With the nonbeliever, (the ONLY neuroosurgeon at our local hospital) I made several calls to docs around the US who agreed that Maddie was exhibiting signs of communicating hydrocephalus. When I gave the nonbeliever the list of doctors names and phone numbers and asked him to call a few of them, he threw the sheet at me and screamed "I don't care if you have talked to God himself! I'm not putting a shunt in your kid!!!" Three days later Maddie was shunted by Dr. Swift. The one downside for us using Dr. Swift has been that he is out of network. The nonbeliever is still the only neurosurgeon at our local hospital, ten minutes away. Dr. Swift is an hour away but we would fly to Egypt for him!

Once a week, my kids have ERT at Cook's and I see the nonbeliever from time to time in the hall or cafeteria or wherever. We snarl at each other and glare and try to keep from spitting on one another. Such ignorance and from a BRAINSURGEON!!

momtoty
March 22nd, 2004, 06:38 PM
I could not get anyone, except the neuro-opthalmologist, to listen to me, until I called Dr Goodrich. I actually had one pedi neuro surgeon ask me if "I wanted him to do brain surgery on my child for no good reason"!
Yes, I've been really bored lately, I thought that opening my baby's head and stomache might spice things up a bit. You know, a change of pace. Can you imagine? :?

Dr Swift is out of network for us too, and we live in Houston so it is quite a drive- but I agree, We would go anywhere to have him care for Ty.

momtoty
March 23rd, 2004, 03:05 PM
Misty,
I agree. :ohno I have not seen him since Dr Swift took over Ty's care. I don't know how I will react when we do bump into each other- which is probably inevidable since his office is in TCH, where Ty sees most of his doctors, and it is also the ER we use.

The Rea Family
March 23rd, 2004, 03:24 PM
Please excuse me for butting in here :lol: , but alot of what i'm reading makes me wonder if Christopher might have this.
He has had an MRI, and CT scan, is it possible that they missed it?
What is a lumbar puncture?
Any help would be appreciated.
Thank you.

momtoty
March 23rd, 2004, 06:48 PM
Carrie,
You are not butting in! It is not inevitable that a child with MPS would get communicating hydrocephalus. Every child is different, and as you know the disease presents itself differently in affected children. I believe the likelihood is higher among MPS 1 and 2 than the others. I am not a medical professional, just a mom. With that said;
If they were doing the MRI and CT scans to check for communicating hydrocephalus, due to symptoms that Christopher is experiencing than the answer is yes- they could miss it in the scans- because MPS children seem to present with very different symptoms and appearence than that of a straight forward case of hydrocephalus. I don't think there are many neurologists and especially neuro surgeons out there experienced with MPS children who also have hydrocephalus.
A lumbar puncture can also be called a spinal tap. This procedure will check for elevated intra-crainial pressure through the flow of CSF in the spine. The child is sedated/anesthtisized, laid prone on a table and then a large needle is inserted into the spine just above the buttocks, this needle has a gauge attached which will give the doctor a reading of the pressure. The normal for a young child should be 10 (or 100mml)or under, it can reach 20 (or 200mml) without too much concern from the doctors, above that is considered high. Unfortunately, this test is not even fail proof, as you may have read in Chase's case. Symptoms (and if the child is under two like in Ty's case) a rapid progression in the head growth are sometimes the only sign. In that case the only way to know for sure is through "intra-crainial pressure monitoring" This is a very invasive procedure, and generally not reccommended unless the child is having very severe symptoms or papilledema(swollen optic nerves) with little else to go on. Many surgeons will not even consider it as I found out with Ty- he had papilledema, high LP pressure, and a very large rapidly growing head- the two NS here in Houston would still not consider it. Luckily, in our case, we found Dr Swift, who has had experience with this type of case and also, when he did a repeat LP- Ty's pressure was very high so he went directly to the shunt.
There is a medicine route you can go- if the pressure is not too high and the child's symptoms are not too bad. Diamox is the medicine- it slows the production of CSF(cerebral spinal fluid) For some children this works well and is well tollerated. Ty tried the diamox last summer when his pressure was 45(450), it helped somewhat for about a month and then his electrolytes got all messed up and he was suspected to have diabetes insipudus. However, once he was off the diamox for about a month- he started to balance out his electrolytes but then his pressure went back up. yikes! this is a long post!
What type of symptoms does Christopher have? how long has he been dealing with this?
I hope this has been of some help to you.

The Rea Family
March 24th, 2004, 07:24 PM
Kim,
Thanks for your response.
CH is not a known problem with Morquio Syndrome.
However, it may not be part of his syndrome, it could be an individual problem.
His symptoms are:
hitting his head on everything,
scraping his head on the floor(we call this carpet sharking),
everytime we go in the car, he will hold his head, and sometimes he will vomit,
also, developmental delay is not a symptom of Morquio Syndrome, and he is delayed by about 2 years.
What do you guys think?
Does this sound like CH?
Thanking you in advance.

momtoty
March 25th, 2004, 12:18 AM
Carrie, Here is an excert from the National Institute of Neurological Disorders and Stroke web site- info on Morquio Syndrome and hydrocephalus: The link is:
http://www.ninds.nih.gov/health_and_med ... bs/mps.htm (http://www.ninds.nih.gov/health_and_medical/pubs/mps.htm)

The excerp is as follows:
"MPS IV, Morquio syndrome, is estimated to occur in one of every 200,000 births. Its two subtypes result from the missing or deficient enzymes N-acetylgalactosamine 6-sulfatase (Type A) or beta-galactosidase (Type B) needed to break down the keratan sulfate sugar chain. Clinical features are similar in both types but appear milder in Morquio Type B. Onset is between ages 1 and 3. Neurological complications include spinal nerve and nerve root compression resulting from extreme, progressive skeletal changes, particularly in the ribs and chest; conductive and/or neurosensitive loss of hearing (see "What are the signs and symptoms?"); and clouded corneas. Intelligence is normal unless hydrocephalus develops and is not treated.


Physical growth slows and often stops around age 8. Skeletal abnormalities include a bell-shaped chest, a flattening or curvature of the spine, shortened long bones, and dysplasia of the hips, knees, ankles, and wrists. The bones that stabilize the connection between the head and neck can be malformed (odontoid hypoplasia); in these cases, a surgical procedure called spinal cervical bone fusion can be lifesaving. Restricted breathing, joint stiffness, and heart disease are also common. Children with the more severe form of Morquio syndrome may not live beyond their twenties or thirties."


Children with Morquio can get Hydrocephalus- it does not seem to be as common as with Hurlers, Hunters, and Martaux-Lamy

I think that if you are concerned about Christopher's head, if he is banging it alot for no other reason, and he exhibits some of the other symptoms- You should persue a second opinion. Ask for a lumbar puncture and we all know (well those of us on this forum anyways) Ct scans and MRIs do not tell the complete story with MPS kids. period. A Lumbar puncture may not even tell the full story- but it is much more reliable that the scans.
Good Luck- maybe you should email Dr Goodrich with your questions? He seems to be quite willing to help as much as he can.
Good Luck.
Kim

Sharon Cochenour
March 25th, 2004, 04:33 PM
Andrew is going to have surgery next month on his sinuses. Basically we can not get his sinus infection to clear up and on his ct scan he has a lot of inflamation and swelling.
His ventricles have always been at the high end of normal. He also has subluxation of C2 and C3. I want to push for a lumbar puncture while he is sedated, to get a baseline reading, due to a higher risk of communicating hydrecephalus, but Barbara said that she thought she heard that they can not get an accurate reading under sedation. Has anyone had a child under anesthesia for their puncture?
I'm putting in a call to Dr. Munzer, but sometimes it takes a while to hear back.
Sharon

momtoty
March 25th, 2004, 06:38 PM
Sharon,
I have been told by several neurologists and neuro surgeons, most importantly, Dr Swift, that the child needs to be sedated for an accurate opening pressure on the LP. Ty was sedated for two and GA for two. They went on to say that it is not like a spinal tap done on an adult, where the paitent needs to be on the side with knees to chest so that the back is streched and sort of rounded because that could give a false elevated reading.Also if the child is crying or tense it could give a false elevated reading. The child should be sedated, completely relaxed, and lying either prone, or propped a little bit on the side but straight not curved.
In each of Ty's 4 LPs, he was out completely- during one of them in the ER he woke up in the middle and I can tell you the minute he tensed up and started to cry that pressure shot up??? They did not take that reading, either. Right before he started to move the fluid was at 45 on the gauge and still slowley rising- They just stuck with 45(450ml) as the reading.
I hope this helps- I would be interested to know what Dr Muenzer's opinion is on the subject.

Sharon Cochenour
March 26th, 2004, 07:11 PM
Thanks Kim,
I thought it would probably be better for him (and me), if we did it during surgery. The doctor is already trying to link with another doctor to do a bronchoscopy and we usually also do an ABR while he is under anesthesia. I was hoping we could also do a baseline LP reading.
Thanks for your input. I let you know what Dr. Munzer says.
Sharon

MelI
March 27th, 2004, 02:52 AM
Hi Sharon,

I'm interested to hear what Dr. Muenzer says as well. Jake had his LP under sedation at Duke while he was having his T & A surgery but I was told at the time that if they were unsure about the pressure or if it was border line or they just weren't convinced it was accurate they would need to do it when he was awake (which of course put me in a panic!) because they can't always get an accurate reading under sedation (similar to what you've heard). Thankfully, his pressure was very obviousely quite high and there was no need to retest but now I'm hearing that they are doing the LP's under sedation routinely so maybe they've changed their thinking on things! :?

momtoty
March 27th, 2004, 12:43 PM
Melanie,
When Ty had his first LP he was 7.5 months old and he was inpatient. He had been admitted through the ER and had been there for 4 days by this time. When the LP was suggested, at first I was told by the attending pediatrician and resident working on Ty's case that Ty would have to be awake for his LP and that it would be very difficult to do on a baby Ty's age. However, when the Neuro surgeon and neurologists were consulted, they said no, we prefer the reading while he is sedated. :? So I have to wonder if there are just some different "schools of thought" on the matter??? I do know, at least in Ty's case, that his pressure shot up when he woke in the middle of a LP, therefore his pressure is obviously higher when he is awake, or atleast, not completely relaxed. I also just remembered something I was told when we were considering the indwelling ICP monitoring. The reason that is so very hard to do on a mobile baby or toddler is because the readings are not accurate if the child is sedated the whole time, and if the child is not sedated it is nearly impossible to keep them from pulling and tugging on the part of the devive outside of their head.
Sharon,
It sounds like, if Andrew had a normal or borderline pressure while sedated, you can't really be sure that is accurate?? :? I think I will pose this question to Dr Goodrich, via email and see what he has to say.

Steve Holland
March 27th, 2004, 11:38 PM
From what we have experienced and have been told, a pressure reading can be somewhat inaccurate under either method. As other have said, if the child is awake and fighting, the pressure will read higher than it normally would. If a child is totally out under anesthesia (like before a surgery), the pressure can vary based on the oxygen saturation level in his blood (contolled and monitored by the anesthesiologist). I know the pressure can vary a lot with a child struggling, but I don't know how much it can vary based on the O2 levels.

If your child is going to be having surgery anyway, my parental advice would be to notify the surgeon and anesthesilogist ahead of time so that they could brush up on the effects of the O2 levels on pressure to ensure that you get as accurate a test as possible.

One issue with a child that is not sedated or only mildly sedated is that it is sometimes difficult to get the LP needle into the proper space, especially with overweight children. Having to dig around to find the space just adds to the length and stress of the procedure. By performing the LP with fluorscopy (like a sonogram), the doc can find the space much easier.

LPs are more often done to test the spinal fluid for diseases like menengitis/encephalitis. So, if your doc doesn't normally test for pressure, it wouldn't hurt to remind him or her that the level of the monometer in relation to the child's body affects the pressure reading. While this would not be an issue if the monometer is directly connected to the needle, it could be if they use tubing between the needle and the monometer.

Due to the problems with getting accurate pressure readings and some docs ignorance of CH, I don't know that the decision to shunt if ever extremely clear cut. (We as the parents were left to make the decision for our three.) However, of the families I have talked to who made the decision to shunt even though they were not 100% sure at the time, I don't know of any that regret their decision. However, I have talked to MPS I families whose children have gone blind due to not being shunted that wish they had. So, that is why this forum and this topic is so important.

Steve Holland

momtoty
March 28th, 2004, 08:01 PM
Steve,
Excellent post. Thank you for your input, it is quite informative, and very important information for any parent of a child with CH or raised ICP of any nature.
And you are so right about shunting being a hard decision. I know that Jim and I agonized over making that decision. Our Dr. gave us his input and reccomendations and then we were left to make the choice. I was in tears about it as the nurse was taking Ty out of my arms to carry him into the OR. However, within 12 hours of the surgery, Ty was a new person. I had such peace that we did exactly the right thing, with exactly the right doctor. and now, only two months later if I had any regrets at all, they would simply be that we waited too long to seek out Dr Swift!

Thanks Again, (Hi to Amy)
Kim Downham