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. 

1. Medications reportedly used by Krabbe patients include: Glycopyrrolate (brand names: Robinol, Cuvposa), Hyoscine patches (Scopolomine).
2. In addition to various medications, some Krabbe children have used a PEP Mask (Positive Expiratory Pressure) or a simple hand operated or mechanical suctioning machine to assist in removing excess secretions.  We were provided with an electric suction machine by Hospice, but rarely used it.  However, some parents rely heavily on these machines for their child’s comfort.
3. We felt like Dylan had fewer problems with secretions because he was still eating by mouth.  He continued the strength of his oral motor functions because he used them all day.  Use of a pacifier also helped because sucking on it caused him to swallow.   

Many Krabbe children experience spasticity or stiffness in their arms and legs. 

1. Dylan did not have a big problem with this for about the first 3 years of his life.  We felt like the physical therapy and massage helped keep this problem at bay for a long time. 
2. When Dylan’s legs eventually became stiffer (particularly in his hamstrings and inner thigh muscles), we found that Botox injections every 3 months (by a pediatric neurologist) made a significant improvement for him.  The injections are made with a very small needle and while uncomfortable at the time, the pain is quick and mild. 
3. In addition, Dylan’s periodic massage and chiropractic therapy was helpful.  We were also able to get our insurance company to purchase a Percussor machine through Dylan’s chiropractor, in exchange for fewer chiropractic visits.  (The “case” we made to the insurance case manager was that instead of coming twice/week, the chiropractor would come once/week and the “payback” was less than 6 months.)  See the following link for the product we purchased:  http://www.impacinc.net/client/index.php?action=show_product_box&page=7) This allowed us, his parents and therapists, to use the percussor as often as Dylan seemed to need it.  
4. We had braces made for Dylan’s hands and feet that helped prevent the feet and hands from “curling” inward.  Your physical therapists can assist in having these braces made for your child.  See Photos #1 & #2 below, that show the braces for Dylan’s feet.  See the Therapies Section and Equipment Section for more information. 
5. Medications used by some Krabbe patients include: Baclofen (Lioresal), Ativan, Gabapentin (Neurontin), Diazepam (Valium).

1. There are surgical options available for severe reflux.  Nissen Fundoplication is a procedure where the esophageal sphincter is tightened.  We understand that some children deal with a lot of gas as a result of this process.  There is a device called a Farrell Valve that may be attached to the tubing while feeding that will allow gas to escape during the feed.
2. Medications used by Krabbe children include the following:  Omeprazole/Prilosec (although an FDA warning exists for this drug indicated prolonged use can result in easier bone fractures in the patients taking the drug), Ranitidine (Brand name: Zantac), Metoclopramide (Brand name: Reglan) (although use for longer than 3 months is discouraged.

Constipation was a continuous problem for Dylan. 

1. We had the best success with Miralax (Glycolax) powder in food/drink.  This kept the stool soft and easy to push out.  It was hard to get the dose right.  A full dose gave him diarrhea, but when we decreased the dose, the stools often got hard.  It was hard for him to push out due to decreased muscle tone. 
2. We used pediatric suppositories for Dylan when his stools got hard.  Sometimes this was unintentional – if we gave him medicine in suppository form, he would often “poop it out”.  This can be a problem with medicine dosage because you don’t know how much his system absorbed.  Sometimes we found it worked best to give him a regular suppository (to cause a bowel movement) before the medicinal one. 
3. Some parents have mentioned using “Lax-a-day” which is polyethylene glycol – it softens the stool by retaining water and is not absorbed by the body. 
4. Many parents have also mentioned using a probiotic (some in powder form) – which can also be provided by some yogurts.  Some parents even blended the yogurt with other foods in a form that was thin enough to use in a G-tube.
5. Parents have also mentioned Keifer, which is packed with probiotics – it is drinkable yogurt and can be found at many natural food stores.
6. Physical stimulation – Gentle pressure of a gloved finger dipped in Vaseline on the rectum can be effective.  Sometimes just taking a child’s temperature rectally can stimulate a bowel movement. 

Chest Congestion is a serious issue for Krabbe children due to increased episodes of breathing difficulty and pneumonia. 

1. We used a hand-held percussor (a little mushroom-shaped blue or pink foam device) on Dylan’s chest and back – often several times a day.  He actually seemed to enjoy the “thumping” on his chest/back and not only does it help with chest congestion, but is also an effective aid in getting a good burp during/after meals. 
2. We saw references to a number of children using a product called “the Vest – Airway Clearance System”, which can be found here:  http://www.thevest.com/patients. 
3. Sometimes breathing treatments are necessary.  This is a medicine in liquid form, which is put through a “nebulizer”, which puts the medicine in gas form for the child to breathe (through a mask).  The medicine varies with the problem.  Albuterol is for asthma patients who are having lung congestion and breathing difficulty.  This was prescribed many times for Dylan and can be used every 4-6 hours when there are problems.  If pneumonia is diagnosed, a steroid is typically used in the nebulizer. 

 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. 

1. We found that if Dylan was just congested nasally, we could put water into the nebulizer, which then made a moist gas.  The moisture helped break up the congestion in his nose to make breathing easier. 
2. Some children also benefited from positioning of the body to assist in drainage.  See this link for examples: http://www.huff-n-puff.net/posturaldrainage.htm.  Some parents also reported that steam from warm shower assisted in improving chest congestion.  (We used water in the nebulizer for the same purpose). 

 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.