Caring 4 Krabbe Kids
A Resource for Families Dealing With Krabbe Disease

Medical Issues

Medical Issues

Nothing in this website should be construed as an attempt to render a medical opinion or an attempt to participate in the practice of medicine.   All topics in this website are merely starting points for discussion with qualified medical professionals of your choosing.  No therapy or medication should be started without first consulting with your child’s doctor.  Information provided here may not be up-to-date, or precise and true and correct.   The information included here is based on a) our personal experience, b) suggestions and/or recommendations from families with Krabbe affected children, and c) from historical information posted on the Krabbe Message Board and the Krabbe Families FaceBook group.  Past successes recorded here are not indicative of future results, nor are they absolutes.  Use any information provided here only with prior approval from your physician. 

What is the Correct Treatment for your child? – Our research indicates that many Krabbe affected children react totally different to specific medications, treatments, etc.  What seems to work well for treatment of symptoms in one child may not work at all for another child.  In fact, there are over 70 different mutations of the Krabbe gene and that number is increasing.  Our goal here is to provide a list of options for discussion with your child’s doctor. 

Considering a Do Not Resuscitate (DNR/DNI) order – 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. 

Traveling Guide – 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. 

Compounding of medications in suppository form – 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.

Pain & Irritability – 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.

Seizure Medications– 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.

Secretion Medications – 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.   

Spasticity/Stiffness – 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).

Reflux

  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. There is also a good website for parents dealing with reflux issues in their children:  http://parent-2-parent.com (click the “home” button on this page and notice the “section widget available for reflux issues). 
  3. 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). 

Vitamins – 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. 

Fever & Temperature Control – Krabbe children often have difficulty regulating body temperature.  We found that Dylan sometimes had “a fever”, with no other symptoms of further sickness.  We used Ibuprofen and Acetaminophen (Tylenol) to assist in temperature control (in suppository form, which was much easier to manage – see section D above regarding compounding of medications).

Feeding Tubes:   - See the “After Diagnosis” section of this website for discussion about our decision to NOT utilize a feeding tube for Dylan.   There are several feeding tube types:

  1. Nasogastric (or “NG-tube”) – is passed through the nostril, down the esophagus and into the stomach.  This type of feeding tube is generally used for short-term feeding.
  2. Gastric (or “G-tube”) – is inserted through a small incision in the abdomen and into the stomach.  This type of feeding tube is used for longer-term feeding.  The feeding tube is generally used where there is difficulty with swallowing and to avoid the risk of aspiration pneumonia. 
  3. Jejunostomy (or “J-tube) – is a tube surgically inserted through the abdomen and into the second part of the small intestine.

Feeding by mouth (WITHOUT a feeding tube) - we elected not to use a g-tube or ng-tube for feeding Dylan.  This is a decision that should be taken very seriously and consideration given to the outcome of this decision.  Dylan was already eating solid foods when he was diagnosed with Krabbe and he continued to enjoy eating.  We chose to feed him as long as possible without a tube for various reasons.  We did; however, spend probably 6 hours a day, or more, feeding him.  We found the feeding process enjoyable, and a great time to just hold Dylan and love on him.  As his disease progressed, we found that altering the normal consistency of the food was necessary. 

Using a blender and food processor, we started to puree all of Dylan’s food.  Testing the best consistency is very important, and the best consistency for your child is likely to change over time.  We often used leftover meals from the rest of the family and provide Dylan with similar food.  We pureed vegetables, fruits, meats and even deserts.   We would puree excess food and freeze in Ziploc bags – storing them flat to minimize freezer space.  We tried to puree a lot of food at one time to avoid doing this on a daily basis.

We also found that Dylan enjoyed flavored yogurts (although he didn’t like anything cold, so we generally had to warm all foods minimally in the microwave).  Yogurt is also a good food to use for dispensing medication.  The flavor in the yogurt tended to cover up the taste of medications.

Liquids – for a number of medical reasons (such as avoiding constipation and dehydration), keeping your child hydrated is very important.  We had difficulty getting Dylan to drink juices (which are quite thin and easy to choke on), but we found a home-made chocolate milk concoction worked well.  We switched Dylan to soy milk at about a year old because he was spitting up so much.  He drank Silk Soy Very Vanilla, which is thicker than regular milk.  We then added: ~1 teaspoon of Nestle’s Quick chocolate milk mix and a serving of Ambrotose glyconutritional supplement.   These powders, in addition to their purported benefits, helped thicken the liquid to make it the right consistency for Dylan.  We also added 1-2 ounces of Heavy Whipping Cream to this mixture.  The cream is good for adding thickness and mega-calories, which helped Dylan continue to gain weight.  We eventually discontinued it because his weight gain was normal. 

Drinking by Bottle -
We found that thin liquids were for difficult for Dylan to swallow without choking or aspirating (getting liquid into the lungs).  The thicker consistency of the chocolate milk mixture worked well for swallowing.  However, a thicker mixture did not work well with normal baby bottle nipples.  We tried buying a number of different ones, but none of those worked.  Eventually, we found that modifying normal nipples by cutting a small “x” over the hole with a knife resulted in the proper size hole for Dylan.  You have to be careful that the holes do not get ripped or stretched, as this could cause choking/aspiration. 

Breathing & Sinus – Dylan often seemed to struggle with breathing, especially when sleeping, and would often “snort”, as though he just could not breathe well enough to stay asleep. 

  1. We found that the stick-on “Breathe Right” nose strips seemed to work well for him and we eventually started applying those each night.  They just open the nostrils a little wider to allow more air flow.
  2. We also found that when he had a cold, positioning him in a recliner to sleep, propped up with pillows and with his head elevated, made him much for comfortable. 
  3. Allergy medications, such as Diphenhydramine (Benadryl) may also help in dealing with sinus issues, and can have an added bonus of making them groggy at night.  However, some children react the opposite to Benadryl and get quite hyper.  In addition, Benadryl can lower the seizure threshold for some children and should not be used if seizures increase.

Sleeping – Although Dylan experienced periods of time where he slept deeply and well, he also sometimes had difficulty sleeping at night. 

  1. Some Krabbe children have indicated the use of Melatonin to assist with sleep.  Although used typically as an antihistamine, Benadryl is also an effective sleep aid and Dylan took this every night.
  2. We also found that Dylan liked to be “firmly” positioned during sleep since his arms and legs were sometimes jumpy – and that would wake him.  We solved this problem by filling long tube socks with beans and laying those on each side of Dylan (against his back and chest) and using them to weight down his blanket (which would in turn prevent his arms from jerking outward and waking him).  Weighted blankets are also comforting to many Krabbe children.  Google the term “weighted blanket” and you will see there are many options/prices.

Inability to Gain or Maintain Weight – Many Krabbe children experience difficulty in maintaining and gaining weight.  We found the best way to get Dylan to gain weight was through the chocolate milk/cream mixture as described in section P, above.  At some point, we were able to cut out the heavy cream because his weight gain was good.  (He weighed about 48 pounds when he died shortly before he turned 5).   Other parents have used Neocate (see http://www.neocate.com) and Similac Alimentum. 

Specific Things to Watch Out For:

  1. Corneal Damage – many Krabbe children have corneal damage from the lack of blinking.  This damage is not reversible, but is easily avoided by apply a soothing suave to the eyes.  Check with your doctor to see what type of ointment and how frequently to use it.  Make sure your child sees an eye doctor periodically to check for damage.
  2. Effects of anesthetic – when reading the accounts of parents on the Krabbe Message Board, we noted that very often a Krabbe child experiences side effects from the use of anesthetic (for surgery, as an example).  Parents reported the children were sometimes less reactive after use of anesthetic and many parents indicated their child was unable to smile after surgery.  We also understand from doctors who study Krabbe children, that many children deteriorated more quickly following the use of anesthesia.  There is no clear understanding as to why this happens, but obviously an area where more study is necessary.
  3. Sensitivity – During a visit to Minneapolis, we found a number of therapists who believe that Krabbe children are often much more reactive to smells, light, and noise than are non-affected children.  They actually recommended a box as a calming resource to tune out annoying lights and sounds.  The theory was that one could construct a 3-sided box (with a top as well – maybe 4 feet off the ground) that would allow your child to sit in a bean bag or baby bouncer seat inside the box, which would limit light and sound.  Although it sounds odd, the idea is to limit sudden noises and changes in light conditions that might upset your child.     We would also recommend trying to determine if your child is annoyed by strong smells.  If so, it is helpful to avoid perfumes and colognes and to ask care-givers or visitors in the home to avoid using such. 
  4. Teething – many Krabbe children experience difficulty when teeth start coming in.  This is likely due to little use of their teeth in chewing hard foods.  You can use a finger toothbrush to massage their teeth and gums to assist the teeth in coming in.
  5. Tooth Care – Dylan experienced issues with brittle teeth and chipping of his teeth.  Because he was fed by mouth but lacked good oral motor control, the sugars from his food and drink spent more time in his mouth than normal.  In hindsight, we would have taken better care of Dylan’s teeth to prevent the damage.  Seek dental assistance to help keep teeth healthy.  Regular brushing, fluoride treatments and massaging the gums will help. 

                                              i.     At one point, we took Dylan to our family dentist.  The dentist had nothing small enough to “prop” Dylan’s mouth open.  This problem was easily fixed by going to a Pediatric dentist, who used a small wedge in Dylan’s mouth to keep it open. 

                                            ii.     See http://www.sageproducts.com for a product that allows the connection of a toothette swab to a suction machine.

  1. Sand-like crystals in diaper – This may indicate that your child needs more fluids.  Check with your child’s doctor first, because increasing liquids could increase secretions or ‘water down’ electrolytes.
  2. Bedsores – from lack of movement – Consider Duoderm treatments and an air chamber mattress to move pressure to various points in the body. See the following link for further information on bed or pressure sores:  http://endoflifecare.tripod.com/juvenilehuntingtonsdisease/i d294.html

Pacifier  - Dylan was a big thumb-sucker before his diagnosis, but became unable to suck his thumb as one of his first symptoms.  He found great comfort in the use of a pacifier, but wasn’t able to keep it in his mouth very well.  We found a simple trick to help him.  We looped a cloth diaper through the handle of the pacifier and used the cloth diaper, bunched up on his chest, to keep the pacifier propped into his mouth so he could suck it at will.  We also felt that using the pacifier attributed to Dylan’s oral motor skills and decreased secretions (because he would swallow as he was sucking the pacifier).  See Photo #3 below, as an illustration of using the cloth diaper with the pacifier. 

Miscellaneous:

  1. Vaccinations – At the recommendation of Dylan’s doctor, we stopped vaccinating Dylan.  However, we were careful to ensure that everyone who came in contact with him was vaccinated, especially with the flu shot, since his immune system was compromised.   We have also heard that if your child is transplanted, you have to be very careful about vaccines – especially those with a live virus. 
Hand Washing – We were very diligent in ensuring everyone coming into the house (including us and our other children and all of Dylan’s medical helpers and therapists) washed their hands – whether or not they intended to touch Dylan. 

 PHOTOS:



Photo #1 - Shows the braces made for Dylan's feet/ankles:




 Photo #2 -- Use of cloth diaper to assist with pacifier



 


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