Questions and Answers
when a child is dying of Batten Disease

Michelle and Sharon, Michelle's last few days

Recently Debra, the mother of a four year old boy, Brandon, contacted me as her son has Down Syndrome, had an AV Canal heart defect successfully operated on, as well as having Late Infantile Batten Disease just like Michelle.  We both know of no others in the world who have been or are in this same situation.  Brandon is sadly very ill in hospital at present and Debra sent me an email just before he went in, asking me the following questions.  She has given her permission for me to put these questions and answers on my home page so that others may benefit.  To e-mail Debra click here
Q.  When Michelle was dying (starving), did she have any pain or anti-anxiety drugs?

A. Michelle had no pain or anti-anxiety drugs when she was dying, only rectal valium to stop seizures.  I don't think she was in pain, I can't think what any pain would be caused by.  I think she was too weak to feel anything.

Q.  I've been in touch with a few other parents and they all say "the feeding tube is best". 
What do you think? 

A. I think a feeding tube is an emotive issue and no one can say what is "best" for anyone else.  It is an individual decision and no one else can judge another's situation.  For me personally, I did not want to prolong my child's suffering or the family's suffering.  Michelle was developing curvature of the spine, had lost just about all of  her vision I think, and was having more and more seizures.  With this disease, I thought "she is going to die, there is nothing I can do but love her and be here for her,  nature has made her lose her ability to swallow, let nature take her now and cause no furthur suffering.  I have to let go sometime, it may as well be now, not in another possibly 18 months, enduring more suffering."  That is just my personal thoughts on the matter and I acknowledge that other people think differently and that there is no right or wrong in this situation.

Q.  Did she contract any disease towards the end?  Brandon has had a sinus infection for a month now that we can't get rid of and he keeps running fevers, even on antibiotics.

A. I have heard of another child having fevers constantly with no apparent reason and not responding to antibiotics although that didn't happen to Michelle constantly.  Michelle developed a high fever and had a cough.  The doctor gave her an antibiotic injection to help prevent pneumonia developing.  I knew she was going to die and told him.....he didn't appear to believe me.  That was a Saturday.  On Monday she spent all morning giggling as if out of control. She could not swallow a drop of water..she would choke on  one drop from an eyedropper.  I was breastfeeding Rachael so I dribbled some breastmilk into Michelle's mouth..and she didn't choke on that!  I swabbed out her mouth frequently and wiped her lips with lemon/glycerine swabs.  We all took turns to hold her. She was peaceful, just having small seizures every now and then.  She died the following Tuesday.

Q.  After Brandon can no longer swallow, how is it going to be for him? 

A.  I cannot say how it will be for Brandon.  I am not even sure how it was for Michelle. She didn't appear to be in pain...she lost half her body mass, she was listless and peaceful, and I'm pretty sure she knew all who loved her were around her, holding her and making her transition to death as loving as possible.

Q. Did Michelle fuss or cry because she knew she was hungry within the first days? 

A. No, she did not fuss or cry.  She didn't appear to know she was hungry.  She just faded. 

Q. If so, how many days was she like that?

A. From the time she got the fever until she died it was 10 days. 

Q.  Was she listless or did she seem comfortable? 

A. Yes she was listless, but she did not seem to be uncomfortable.

Q.  Before she stopped swallowing did she begin to decrease her food intake on her own? (I think Brandon is very dehydrated even now.  He has one wet diaper a day and refuses to drink most of the time.  He gets fluids from applesauce and the other foods he eats (which he doesn't eat much of any more).  He still gets fussy when he is hungry but at least one meal a day he doesn't fuss.  He usually skips lunch or dinner on a daily basis.  I've learned if he doesn't fuss, he's not hungry.  He'll turn his head if we try to feed him when he isn't hungry.)

A. Yes...she very gradually decreased her food intake..her ability to swallow decreased very gradually too, and when she got the fever, her swallowing stopped altogether.

Q.  Will he slip into a coma-like state and just be.....there? 

A. I cannot say what Brandon will do.  Michelle did not slip into a coma-like state, in the true sense of the word.  She seemed to be not "with it" some of the time but had some level of awareness most of the time.  I'm pretty sure that she knew we were there, loving her.  She raised her arm to her Dad's chest about 10 minutes before she died and I could swear she was looking at us. 

Q.  He is having a lot of myoclonic jerks now and keeps his arms close to his body with fists clenched and laughs all the time in his sleep.  He sleeps about 4 hours a day now.  He can no longer sit up at all. I think I will be at peace when he goes.  As hard as that may sound, it will just be sad, depressing relief.  Does that make sense? 

A. Yes, that makes all the sense in the world to me.  I was simultaneously relieved it was over but sad that she had gone.  Three years and nearly 8 months down the track I am still relieved that she has gone and sad that she has died, Michelle could no longer sit up at all.  Watching Michelle decline like that was what helped me to want her to go and to start learning to let her go (not a thing that happened overnight for me).  I pleaded with God to take her, I begged her to fly away like a butterfly and in the end, I said "Go to the Angels" and she went.

Here are some questions that I asked that are not covered above:

Q.  Do you think that Michelle having Down Syndrome affected your decision regarding a feeding tube? 

A. I tossed that idea around in my mind a lot.....she never talked or ran or did many of the things other children did...but she communicated, had fun, was full of joy...and she lost all of that just like any other child with Batten Disease does.  I don't think it made any difference...I never had a child with Batten Disease without Down Syndrome, so it can't really be something I can compare to the situation I was in.  During the time when I wondered if her little sister also had Batten Disease, I wondered if I would make the same decision about a feeding tube for her..and concluded that there was no point in thinking about it unless I was in that situation.  We can't surmise about situations that don't exist for us. We have to wait until we are in them to know what feels right.

Q.  Michelle has raspy, noisy breathing, which keeps stopping.  I think she's gone, then suddenly she takes a big gasp and continues.  What is happening?

A. Michelle is Cheyne-Stoking.   This means that she is going to die very soon, probably within 24 to 36 hours.  (That is what happened.)

Q.  What do I do when Michelle dies if it is during the night?  Do I call a doctor?  Do I close her eyes?

A. When I could see that it wasn't going to be long before Michelle died, I phoned the funeral directors who assured me if it happened overnight there was no need to call anyone, to just phone as soon as possible in the morning.  Michelle died at 3.20 a.m. and I tried to close her eyes like I have seen on TV.  It didn't work!  It was quite unnerving.  We took some photos of her and left her on our bed, then went to sleep for a few hours.  We were all exhausted.

Poems about when Michelle was dying

A brief story about when Michelle died....

A summary, with photos of Michelle's entire life
(please be warned two photos on that page were taken after she died)

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Batten Disease Information   -   BDSRA

The BDFA Homepage (Batten's Disease Family Assoc. UK)

Down Syndrome Information

Children's Heart Society

Epilepsy Information

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Page written July 2000

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