Hydrocephalus

We have a dear friend who has a seven year old son with hydrocephaly. He was diagnosed with it in utero but my friends went ahead and had the baby anyway. Their son Zachary has additional complications from the hydrocephaly including optic nerve hypoplasia (resulting in limited vision), adrenal gland problems, etc, etc. My friend actually started a foundation for her son called One Small Voice which raises research money for ONH and hydrocephaly. If you go to the website (onesmallvoice.com) you'll get more information on what Zach and other kids like him face. His mom, Debbie is an amazing person who would be happy to talk to other parents facing a similar situation and can guide you in terms of what to expect, special schooling and care, etc. Just e-mail her through the website and she can probably help a lot. We just saw Zach last weekend and it is definitely a tough road. I wish you all lots of luck and support with this child.

Our son was diagnosed with hydrocephalus at birth and given a shunt at 4 months old. We belong to a hydrocephalus support group in Chicago which has been an enormous amount of help and support. I would strongly recommend your family seeking out similar support, whether it be through the internet or locally. Hydrocephalus has a wide range of how people experience it... some people are lucky enough to hardly ever have a shunt revision, others have so many that they look into other options like an ETV. Being well informed about what to expect really helps. There's a book called Hydrocephalus: A Guide for Patients, Family, and Friends is a fabulous resource to help get a better understanding of what to expect from a medical, health, and social perspective. Best of luck to you.

M. K

Yes, I have seen VP shunts a lot when I was a PICU nurse. Unfortunately they often get infected or malfunction and have to be replaced. I'd recommend calling some of the pediatric hospitals and ask about a support group--I'm sure Children's has one.

My 5 year old niece also has hydrocephalus. She had the shunt put in when she was 3 months old and has not had any adjustments since. Your sister/brother should probably take your niece to see another doctor. It may seem like a lot at this point as I'm sure they feel that they have invested a lot of time in their current doc; however, if a new doctor could prevent any permanent damage your niece may sustain as a result of all these adjustments it will be well worth it.

If she's not already, your niece should be seeing a pediatric nuerosurgeon (as opposed to a general nuerosurgeon). My niece's doctor is at Children's Memorial. Also, I've heard there is a very highly regarded pediatric neurosurgeon at the University of Chicago -- I think his name is Dr. Frim. I think he specializes in shunts and has actually developed a type that works for some kids when a regular shunt does not. (I have not heard good things about Loyola.)

My father was diagnosed with that disease 4 years ago. He had the shunt put in and made an improvement. He suffered for years with strange symptoms but within a day of being at Mayo Clinic in Rochester, MN he was correctly diagnosed. I am not sure where your niece lives but they might want to think about going to Mayo Clinic for an opinion. I have also heard that Dr. Chirillo in Chicago is excellent. Once the shunt is working properly your niece will make a great recovery. Will keep you and your family in my prayers.

I know how stressful it can be having a child in and out of the hospital all of the time. I am parenting a child with special needs who is also medically fragile. We have not needed to have a shunt put in but we have had our share of surgeries, emergencies and illnesses that have required extensive hospital stays.

Please know that that you and your niece's family are not alone.

I happen to know someone who has a child with this but the child is in his 30's now. It wasn't a real good situation back then. I hope this is better for people now with this problem.

Hi Jackie!

My son, Gabe, does not have hydrocephalus. He does however have a programmable shunt. Gabe has craniosynostosis, chiari malformation and pseudo tumor cerebri. To treat the PTC he has a shunt. His shunt is a lumbo peritoneal (LP) shunt. It goes from the lumbar region of his spinal column to his belly and drains the excess CSF there.

Programmable shunts are wonderful. Before programmable shunts, every time they had to adjust how much a child was draining they would have to go in and surgically change out the components of the shunt. Thus lead to an increased risk in infection (meningitis) and more scar tissue. I believe that there are only 2 types of programmable shunts on the market right now, I could be wrong though. Programmable shunts allow the shunt to drain more or less CSF by “reprogramming” it. Our shunt uses a programmer with a large magnet that plugs into the wall. We are able to place the transmitter over the dial and reprogram Gabe’s shunt to a higher or lower number. Our shunt it set up with the higher the number on the programmer the less fluid that is drained off, lower the number the MORE fluid that is drained off.

At least for PTC when they insert the shunt they set it at a certain level. For us they wanted us to wait a month before we started adjusting the pressure so that Gabe could accommodate to the shunt. After the first month we then started adjusting his shunt with the programmer. We are able to do ours at home as we have a programmer. (I am also a nurse) Some doctors want the kids to come into the clinic to have it done. Older kids are able to be a little more verbal and if they are not verbal they are physically doing more that allows the parents to gauge how the shunt is working. In a baby that can be hard.

I would assume that they want the baby to come to the hospital so that they can watch how she responds to the new pressure setting. A change in pressure can cause low pressure symptoms which are worse than high pressure symptoms. Finding the right setting is a slow process. It is one of many “ups and downs” and I can only imagine in a baby how much more difficult it is to figure out. My son is able to tell me and is able to figure out what is high pressure and what is low pressure. Do we need to go higher or lower on the programmer. We are very fortunate to be in close contact with our neurosurgeon via email at all times also.

Unless your niece is having complications that is putting her into the hospital post re-programming I would try not to worry. For a baby to assess for how the shunt is working is a watch and see what she starts doing. Meeting milestones, ect.

Good Luck to your niece.
M.

Edited to Add:
We also use Dr. Frim. One of his passions is hydrocephalus. He uses a lot of the programmable shunts.

Hi, My dd was diagnosed with a Grade III brain bleed/hydro when I was 32 weeks PG.
At 5 months old she ended up needing a shunt, but at the last minute they decided to try a 3rd fenestration (ventriculostomy). Knock on wood it worked.
I have heard that with shunts(or any apparatus)the chances of a lot of revisions is possible ......as the PICU nurse mentioned.

How old are your b/g twins now? Mine are 5.

ETA~ I second Joanna on Dr Frim. (Both of my twins surgery was done at U of C~my ds was cardiac though).
I loved Dr Frim! I brought my dd in, they admitted her that day & did surgery in the AM. The only problem was, DR Frim was on vacation, but his associate/partner Dr Yamini was the one who recommended the fenstration vs the shunt, & he did the procedure. He was just as awesome. I highly recommend them

Jackie,

My dd was diagnosed with hydro when I was also pregnant, they put the shunt in when she was 6 weeks old and knock on wood she hasn't had any issues with it at all. She is now 16 months old. Your neice is probably experiencing draining issues or infections from it, these are the 2 main reasons within the first 6 months - year of revisions. I can't say anything but good things about Children's memorial and Dr. Dipatri. He works with the Dr. Tomita as well. They are both Professors and Dr. Dipatri has a great bedside manor. If anything call them to ge a second opinion they are always willing to do that. I recently had a friend take her MRI's there to review for a different situation (tumor) but they were able to properly diagnose it.
Here is a little info on Tomita: http://www.tomita.yourmd.com/
He is a good dr. but I personally like dipatri as you can understand him better and they all work together if there is a problem. Good luck and keep us updated.

You should ask the dr. why honey but it probably has to do with the childs growth causing movement of the valve or something like that. Just check with him/her to explain the reasoning for it honey. Don't ever be shy or feel foolish about asking questions 100 times if necessary when it comes to your child. Good luck mommy

They discovered my nephew's cyst during an ultrasound when my sister was pregnant. He had a shunt (drain) installed the day after he was born. The first two years or so he was in and out of the hospital quite a bit to have shunt revisions. He is now almost ten and has not had a surgery for years.

Jackie B -
I have a friend who was diagnosed the same way, however she is now 30 years old! She has had many, many VP Shunt revisions and I know she would be more than happy to speak with you or your family.
Keep your chin up - our prayers are with you and your family.