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