Nothing in this website should be construed as an attempt to render a medical opinion. All topics in this website are merely starting points for discussion with qualified medical professionals. No therapy or medication should be started without first consulting with your child’s doctor.
A DNR is a legal order to respect the wishes of a patient (or guardian) to not undergo CPR or advanced cardiac life support if the patient’s heart were to stop or they were to stop breathing. The DNR does not affect any treatment other than those that would require cardiac compression. DNI (Do Not Intubate) deals with intubation (insertion of a tube into the windpipe to serve as a conduit for a ventilator) and may sometimes be a temporary need (for example when a child has pneumonia). Regardless, families need to be prepared for these decisions as they are often decisions that must be made urgently during a medical emergency.
If your child is under the care of a hospice organization, they should be able to assist you in making the decision as to whether you want to sign a DNR/DNI form and the process to complete such a form. If your child is not under hospice care, we would suggest a Google search for “DNR Order in (your state name)”.
The decision to sign a DNR order is not one you will want to make when you and your child are going through a medical emergency. The decision is very personal and emotional and better made under non-emergency conditions. Without a DNR, it is likely that hospital personnel will do everything in their power to “save” your child. Our personal belief is that a decision to withhold a ventilator, for example, is a very different decision from allowing the placing of a ventilator and then deciding at a later date to remove that treatment. In other words, it is easier to not start “heroic measures” than it is to terminate them once they are started.
You should create a traveling guide for your child to be taken with you any time you travel outside of the reach of your doctors and/or hospice organization. Items to include: medical records with a history of previous hospital visits and test results, lists of all current medications and dosages, the names and contact information for all of your doctors, therapists and hospice. You should specifically ask your doctors who to call if you have problems when out of town. You should also prepare a list of local children’s hospitals in areas you plan to visit and ask your local hospice organization if there is a related group in the area you are visiting.
Our hospice organization was very proactive in identifying a pharmacist that was able to provide us with a number of medications (Ibuprofen, Acetaminophen, Seizure medication, etc.) that could be provided in suppository form. We found these medications much easier to give to Dylan than any type of liquid/oral medication.
Doctors do not understand why Krabbe babies are often so irritable. It is possible the pain originates from the peripheral neuropathy, possibly “central” – but not really pain as we normally experience. However, it is common that over time the irritability declines and sometimes disappears entirely.
1) We found that physically holding our son was the best comfort when he was irritable and experiencing pain. During his waking hours, especially during the first 18 months after his diagnosis, we held our son for the majority of the day.
2) Other medications used for pain and irritability by various children: Lorazepam, Gabapentin (brand name: Neurontin), Morphine, Baclofen, Tizanidine (brand name Zanaflex), Phenobarbital, Codeine, Ibuprofen, Paracetamol (Acetaminophen), Clonazepam (Klonepin), and Atarax.
3) One parent recently mentioned their success in using Nabilone, which is apparently a synthetic cannabinoid that is for nerve pain in MS patients and also helps with nausea and irritability in pediatric cancer patients.
4) Some parents have also had success by giving their child an ounce of cooled chamomile tea.
5) A number of parents have mentioned that when their child was in pain and irritable, that holding the child seemed to worsen the pain for them. Several of these same parents have mentioned that in the same way we “shake” our arms or legs when they fall asleep, they do the same for their child. The description was as follows: “loosely grab the ankles and shake vigorously”. They also mentioned using a “vibrating pad” to lay their child on.
We noticed that our Dylan lost his ability to smile shortly after he started taking seizure medication. We discussed trying a different medication with our doctors, but they felt the loss of his smile was disease progression rather than medication related. After a period of months, we asked our doctor to try a different medication and Dylan got his smile back. (The smiles were not frequent and kind of crooked, but we were blessed to have them.) Do not hesitate to ask your doctor about trying different medications. The loss of Dylan’s smile for months and knowing we could have done something to change that at an earlier date was one of our few regrets in treatment choices. The choice of medication will depend on the type of seizure as well as the age of the child. Medications used by a number of Krabbe children include: Phenobarbital, Gabapentin (brand name: Neurontin) Clonazepam, Keppra, Topiramate (Topamax), Oxcarbazepine (Trileptal), Lamictal (although there is apparently an FDA warning that Lamictal can cause meningitis and can also cause serious drug rashes as well). Diastat (taken rectally) is also used for short-term treatment of seizures in patients already taking other seizure medication. We did use Diastat several times with Dylan to break a chain of ongoing seizure activity.
Many Krabbe children struggle with excess secretions that make breathing and eating difficult.
Many Krabbe children experience spasticity or stiffness in their arms and legs.
Constipation was a continuous problem for Dylan.
Chest Congestion is a serious issue for Krabbe children due to increased episodes of breathing difficulty and pneumonia.
i. Standard electric nebulizers are used in most situations. They plug into an outlet and are quite heavy and loud.
ii. Dylan was prescribed with a battery powered nebulizer. We found this greatly aided in his comfort. The electric nebulizer made his startle reflex react when you turned it on, and the noise kept him on edge for the whole treatment. With the battery powered nebulizer, he barely knew it was happening. Also, it was tiny and very easy to travel with. Another benefit is that treatments can be given on the road, if necessary.
If your child is fed by tube, their nutrition is likely to be quite adequate. If they eat by mouth, adequate nutrition is harder to ensure. It would be a good idea to discuss with your doctor what your child actually eats to see if there are deficiencies which can be made up for with vitamin and/or nutritional supplements. We used Ambrotose by Mannatech, which is a glyconutrient dietary supplement in powder form that we mixed into Dylan’s milk. Many people give this product rave reviews. For us, it fell into the category of “it is natural, can’t hurt and might help, so let’s try it”. This is one of the few things that was quite different about Dylan’s treatment from most Krabbe children. Could it have attributed to his length of life? We will never know for sure, but possibly.