Medical Issues

 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:

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.

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. 

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.

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.

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.  

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

 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.