7 year old with adrenoleukodystrophy

  1. My neighbor's son is 7 years old and dying of adrenoleukodystropy. He is now nonambulatory, blind, hearing questionable, cannot talk, having severe muscle spasms - currently treated with Valium prn. He has a neurologist at Los Angeles Children's Hospital but because of HMO things are slow. The big issue right now is swallowing and nutritional and fluid intake. I have spoken to the family and to the nurse at the doctor's office about this. Family is not ready for Hospice. They want to insert a G-tube for nutrition but are running into delays with getting this scheduled. The child has pulled out NG tubes in the past and the family does not want to put him through that again. I currently am an RN working with ADULTS in home care. Last pediatric patient I had was in nursing school. Does anyone have any information in terms of care that I can pass along to the family?
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  2. 8 Comments

  3. by   live4today
    As difficult as it may be to let go and let God......that would be my advice to this family, or any family in their situation. They need counseling to help them cope with their son's illness, and his pending death. They could also benefit from a support group in the town they live in. I'm sure there must be one near them...perhaps Hospice or American Red Cross would know of one to recommend to them. Research their son's illness on the web, and see what insights those websites may offer you to share with the family. Letting their son die with dignity and being held by them so he feels the warm love from his parents arms is most important to him right now. Ask the child what he wants....many children of seven years of age are living with terminal illnesses every day, and you'd be surprised at how they view dying at their tender ages. God prepares them for their going home to be with Him. They just know....:kiss
    Last edit by live4today on Jul 14, '02
  4. by   Judy, RN BSN
    Thanks for the advice. I have spoken to them about Hospice and there is a wonderful Hospice in my area that cares for terminally ill children. Cognitive function is affected. He can't see, speak, hearing questionable, so unfortunately it makes understanding what he is feeling/thinking difficult, other than the obvious pain when his muscles spasm from the disease. I have already spent time on the web regarding his disease and have made copies of all the information I could find. It's hard to watch a kid die from this. At this time I am helping the family with comfort measures as much as possible.
  5. by   misti_z
    What a hard thing for that family to go through.

    I will keep them in my thoughts.
  6. by   live4today
    Judy......why not go ahead and add this family to the Prayer Chain Thread Hoolihan started so we can all be praying for them. You are such a blessing to that family, and so I will pray for you as well. :kiss
  7. by   kids
    Originally posted by Judy, RN BSN
    The big issue right now is swallowing and nutritional and fluid intake.
    edited down by kids


    Hey! A question that is right up my alley!

    First things I need to know is...

    Is his gag intact?
    How high is his risk of aspiration?
    Does he have a suck reflex?
    Is he interested in oral feeding?

    Yes, I have some ideas, but can narrow it down with more info.

    -nancy
  8. by   Judy, RN BSN
    Thanks all again for your responses. Nancy - the family has taken the child to emergency room at Childen's (where his treating physician is) today. The hospital has admitted him and is currently giving IV fluids. The family states the
    doctor will keep him hospitalized until G-tube can be placed. I post updates as I get them. Judy.
  9. by   kids
    Thank God.

    I was pulling up all my feeding stuff to prepare to respond based on your answers.

    We have an I have an 10 yr old on my caseload with a disorder that has never been given a name...the best guess is it is one of those bizarre unknown fatty acid metabolism by-product disorders. She is being bottle fed 50:50 Nutrin Jr/Boost. The only oral instinct she has left is suck & swallow.
  10. by   Judy, RN BSN
    Update on this case: the boy died at home on November 16 with his mother by his side. This is one nasty disease.

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