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"Research" Results

Comparison of Hydranencephaly Survey Results

We have conducted 5 surveys regarding Hydranencephaly over the years.  I’ve included the results of each of the surveys.  Survey # 1 was conducted in 2001 on an online survey service. It was conducted over approximately 12 months and had 58 participated. The 2^nd one was conducted using the online polls on the Yahoo groups site. Each question had a different number of participants. Only people subscribed to the mailing list were able to participate, and those who had more than one child with the condition could only answer for one child. Therefore I don’t think it’s as accurate. The 3^rd was conducted online via a form and via snail mail and was available from September 2002-February 2003. 81 families participated. The 4^th and 5^th surveys were each conducted on a different online survey hosting site over the period of about a year. The 2006 survey was extremely long and covered a wide variety of questions. For the most part I have just included the questions that in the other surveys. A more complete look at the results of this survey will be done in separate documents. The 2010 survey was much shorter and designed to get information on the most common issues faced by children with Hydranencephaly. As there were differences in how questions were phrased and what answers were provided between the surveys it was sometimes hard to draw conclusions and provide a clear result.  As I no longer have the original results of the earlier surveys I have no way of knowing which children participated. Many of the same children  are represented in each survey but there were others  whose information was provided in only one survey.  These surveys are the most comprehensive compilation of information about children with Hydranencephaly. It's is our hope that this information will help others change their attitudes and care of our children.

General Information
Mother’s age:

Our numbers on mother’s age have gotten the attention of Marc Lubinsky as although the percentage of mothers who were over the age of 20 is greater, the higher numbers of mothers under the age of 20 is higher than what would be expected statistically. He is the author of 2 articles online which can be read at: http://www.hydranencephaly.com/causeofhydran.htm. They have been on both our website and in the books. I had discounted his results due to the fact that there were a higher percentage of mothers over 20. But as he pointed out:  “there is no question that there is an increased rate in younger mothers. Mothers under 20 make up about 10% of all births, so they would be expected to have roughly the same % of children with Hydranencephaly- instead, the sample from your survey had 41%, clearly an increase! Also, of all teenage births, about 1/3 are to women under 18 years old, but those women had 73% of all children with Hydranencephaly born to teenagers!  In other words, the effect is greater as mothers get younger.”

He plans to publish an article on this topic at some point in the future.

Did mom have prenatal care?

In the 2001 and 2003 surveys the ratio of those who had and those who didn’t have prenatal care are similar. However that is drastically different in the 2006 survey. I don’t know a reason for that.

Suspected reason for Hydranencephaly condition

The most common cause given seems to be a prenatal stroke or that the cause is unknown.

Health Issues
What are your child’s most common health concerns?

*number is greater than # who took survey as some people checked more than 1 category

I question the validity of the answers to this question but leave it in so people can see the variety of health concerns a child with Hydranencephaly may have.

Does your child have allergies?

What is your child allergic to?

Does your child have Hydrocephalus?

Does your child have a shunt?

* all but 3 of the children in the 2010 survey who had hydrocephalus, had shunts.

Respiratory Issues
Does your child have/ use any of the following?

*Bipap & ventilator use combined in 2006 survey

Other Health Issues

*Premature Puberty question was asked in a different manner in the 2006 survey. The results are as follows:

Precocious (Premature) Puberty is described as:  the onset of signs of puberty before age 7 or 8 in girls and age 9 in boys — can be physically and emotionally difficult for kids and can sometimes be the sign of an underlying health problem (http://kidshealth.org/parent/medical/sexual/precocious.html )

So, with 80% of the children represented here reporting puberty as starting before 10, the incidence of early puberty in children with Hydranencephaly is quite high.

Has your child had bacterial or viral pneumonia?

Has your child had Aspiration pneumonia?

Is your child more likely to have a

*”normal” is the category asked in this survey
**Question was: Does your child have trouble controlling his/her temperature: Yes: 75 (71%), No: 31 (29%).  The child’s common temperatures were given as an open ended question and are varied.

It seems that more children have lower temperatures than higher.

Does your child have trouble sleeping?

I find the results of this question interesting. From what parents say, all the children have trouble sleeping. But this shows that just over half the children have trouble sleeping.

Does your child take any medication to help him/her sleep?

Have you tried Melatonin?

*the use of melatonin was included in a general question about what medications a child had used for sleep.

In each of the surveys (2001-2003) only 1 person indicated that Melatonin had worked for their child. In the 2006 survey 2 people indicated Melatonin worked best for their child. As you can see it doesn’t work as a rule.

  Is constipation a problem for your child?

Does your child take medications for his/her constipation?

What medications has your child taken for his/her constipation?

This is a hard question to get results for. I have no other info on the 2003 survey so have no way of knowing how many children are represented.  In the 2006 survey all participants just clicked on meds they’ve used for their child. This does not represent how many children participated either.  But you can see that families have tried multiple medications to help treat constipation in their child.

The 2006 survey asked which of these worked best for their child. It was a choose one answer.  The results are as follows:

Seizures and related issues:
Does your child have seizures?

Does your child have “coma” incidences (where he/she sleeps for several days and is hard to rouse)?

No other seizure related questions were asked in the 2001, 2002, 2003 and 2010 surveys. However the 2006 survey went in to much more detail.  All the seizure related questions in that survey will be covered in another document. For information sake I’m presenting a few of those questions here.

Does your child drift into “absences?”

Do these happen?

Is it easy to tell when your child is "back"?

Have your child’s seizures been picked up by a EEG?

Can your child be brought out of a seizure by a touch or other stimulus?

Can a seizure be brought on by something external (position, noise, lights, stress, etc)?

Feeding
Does your child have a feeding tube?

At what age did your child get his/her feeding tube?

Although there doesn’t seem to be a clear age by which most children who get a tube have it, over 50% (2006: 54%, 2010: 65%) get them from 1 month to 2 years.  This makes sense as in most cases the children do relatively well for awhile eating by mouth. As they get older it becomes harder on them.  Most of the time children are given the opportunity to eat orally at first.

How is your child fed?

It’s too bad we didn’t ask “Does your child have a feeding tube?” in the first 3 surveys. I think more children are getting feeding tubes now than in earlier days but that isn’t shown clearly in the questions “How is your child fed?”

Has your child been diagnosed as aspirating?

Apart from the 2010 results, it seems that fewer children are diagnosed as aspirating. But it’s quite close so we can’t say clearly either “all children with Hydranencephaly aspirate” or “most children with Hydranencephaly don’t aspirate”. We can say it’s fairly common that they do aspirate.

Does your child have Gastroesophageal Reflux disorder (GERD or reflux)?

This shows that over 50% of children with Hydranencephaly have difficulties with reflux. This is a very painful condition and accounts for a lot of the discomfort children have in their first months.

Has your child had a Nissen Fundoplication?

The following 3 questions were only asked in the 2006 survey.

Did it (Nissen) work?

Has the Nissen needed to be repeated?

 Has your child had a Pyloroplasty? 

This is an operation where the bottom of the stomach is opened up to allow faster drainage It’s often done at the same time as the Nissen. But from these numbers, it appears that it’s not done as routinely with the Nissen as it has been in the past.

If your child eats orally, does he/she manage liquids?

The question was asked differently in the 2006 survey.  Results follow.

Do you thicken liquids for your child?

The following question was only asked in the 2006 survey:

 If your child eats orally, what textures of food can your child eat?

Physical abilities and issues
What is your child’s tone (how loose or stiff the body is)?

As you can see, children with Hydranencephaly tend to have either high tone (stiff) or a mixed tone (both stiff and floppy). I observed this in my child as well.  High tone is the cause of a lot of orthopedic problems faced by the children.

Has your child received Botox or phenol injections (used to help reduce tone)

These injections are done in muscle groups, most often in the legs or arms and have to be repeated every 3-6 months. In many cases they can either prevent the need for surgery or tell the dr if surgery will work on a particular child (if paralyzing the muscle stops the tightness, surgery that cuts the tendon will also work).  It’s use in children has increased over the last few years.

Has your child taken Baclofen to relieve spasticity?

Did your child take Baclofen orally or via an implanted pump (Intrathecal)?

It’s been suggested that children may have an increase in seizures when taking Baclofen. The following question was only asked in the 2006 and 2010 surveys.

So in the children with Hydranencephaly this doesn’t hold true for a majority. And in some cases the benefit of the Baclofen has outweighed the risk of increased seizures.

Can your child roll?

Can your child move his/her hands/arms

Can your child move his/her legs?

 How is your child’s head control?

These results aren’t great as the same questions options weren’t given in the 2006 survey. However it does seem to say that the majority of the children have poor or little head control. This is something all of us have worked on with our children.

Can your child touch or manipulate items?

Again, as the “a little” question wasn’t included in the 2006 survey, the numbers don’t give us a true picture of what the children can do. But, if you combine “yes” and “a little” in the 2003 survey you get 38 (49%). So in that survey more could touch or manipulate items than couldn’t. In the 2006 survey vastly more couldn’t touch or manipulate items than could.  Different children participated in each survey with some overlap.

Can your child pick up items?

 These results show that although many children can touch or manipulate items few can actually pick them up.

Can your child bring his/her hands to midline?

The following questions on physical abilities were only asked on the 2006 survey.  I am presenting only the question. Much more was asked in the survey and will be included in the 2006 survey report.

Can your child walk?

Can your child sit unassisted?

Can your child sit while supported by legs only?

 Can your child crawl?

 Can your child move on the floor other than crawling (by kicking his/her legs, using his/her head to help move)?

Orthopedic concerns

Has your child had a dislocated hip?

Has your child had a subluxed hip? (partly out)

*subluxed hip was included as part of the question about a dislocated hip.

Has your child had hip surgery?

Does your child have scoliosis (curved spine)?

 Does your child have Kyphosis (hunched back)

 Does your child have any contractures (limbs that can’t be straightened out)

 Does/did your child wear/use the following equipment?

*does not include number of children wearing AFOs
As I don’t have the earlier surveys I can’t tell exactly how the percentages were achieved. However it seems that a larger number of children use AFOs (ankle foot orthotics) and hand splints during the day. More also use a stander.

Social/Emotional attributes
Can/does your child give hugs and kisses?

 Can/does your child cry?

 What is your child’s general mood?

What was your child’s mood as a baby?

 As you can see there is a major difference in the mood of a child in his first year and then in later years. The percentage of children who are irritable during their first year actually increases in the last 2 surveys. The percentage of children who are happy over all also increases dramatically in the last survey. We use this info to comfort families who are dealing with constantly fussy babies. It does get better.

Is your child aware of his/her surroundings?

 Is your child aware of objects?

These numbers show that children with Hydranencephaly are indeed aware of their surroundings and objects. More details about this aspect of the children will be looked at in depth in a document called “Cognitive Development and Awareness in Children with Hydranencephaly” which should be available by September 2011.

Does your child have a favorite toy?

Does your child have a security object?

Does your child attend school?

What type of program is he/she in?

Vision/Hearing

Can your child hear?

Can your child make any sounds?

Does your child use any words meaningfully?

Does your child use a communication device?

Can your child understand what is said to him/her?

Will your child echo you?

Can your child see?