Casey

his is the story of Casey, a little boy with RDEB who has a trach. 

The story is written by his mother.

Casey had his trach put in in July of 2008 when he was 14 months old. Looking back, there were symptoms that we should have recognized as EB airway issues, but we were continually told, even by the best EB people in the country that dystrophic children do not get severe airway issues...Since a few months old, Casey would have episodes of gagging, then spitting up/vomiting blood and a piece of skin would actually slough off and come out...The key to the airway issue here was immediately following he would go into almost a week of stridor (noise on inspiration)...He lungs would be clear..we'd use nebulizers, steroids etc..but nothing worked ..it had to work itself out over a week or two time span. Every doctor and the EB clinic we see told us it was a blister in the esophagus and the skin from it is most likely flapping into the trachea at the point of the epiglottis..we believed it as it did make sense..Noboday wanted to do an invasive scope to really find out..we agreed..Until it happened several other times and we really did not feel in our hearts that the stridor was from the skin..esp..since the last several times, the skin came out?? They still said that a small piece must have remained..we were beginning to get skeptical but they were the experts...Another key symptom ...Casey drooled so much, he could soak a shirt an hour..again we were told...teething...It ended up his epiglottis was so swollen that he couldn't swallow the extra saliva...

In July of 2007, Casey had developed pneumonia..He was on antibiotics and John went to give him his medicine and he vomited, coughed and could not breathe...at all..he turned blue eventually, eyes rolled back and he took on a typical posture of a child who was not breathing..we sat him up, thought he was choking, banged his back repeatedly and he began to shallowly breathe..we took him to the local hospital by ambulance who gave him steroids, nebulizer treatments, etc..and had no idea what had happened as he remained lethargic, breathing shallowly and using accessory muscles to breathe...and again..stridoring...we transferred him to another hospital where John works and his doctors are all affiliated...the pulmonologist came in immediately said something is blocking his airway and he needs to be scoped...we called in ENT but they decided they were not capable of doing the test as they were unfamiliar with EB so since Casey had been a patient at cincinnati children's hospital before, we were told to transfer him there..so on the 7th of July Casey was transferred to children's hospital by medical flight where they did the bronchoscope and found out that he had an airway smaller than the size of a straw...he must have coughed up some mucous at home that got lodged in that tiny swollen airway ..his epiglottis was so swollen it almost completely blocked his airway..So, they placed him on a vent after the scope and two days later when an OR opened, they placed the trach without complication. Since that time, Casey has had a bronchoscope every 8 weeks that remains clear..his epiglottis just 8 weeks later, was almost completely healed much to their surprise and has remained that way since..of course he HAS had two episodes of blistering since then at home so we know it is continuing...I just didn't know if it was causing a blocked airway again because now we had the trach..the last scope we had done was good...and they discussed the option of taking the trach out...we opted out of that choice and decided to cap it instead..this way we could monitor him at home and we would know if the next blistering episode resulted in airway complications or did his airway grow bigger to allow a blister and swelling and not cut off the airway..The cap is just a small plastic cap that goes over the hole of the trach allowing him to breathe "normally" passing air through his upper airway..restoring his sense of taste and smell and normal speech...It worked beautifully and casey tolerated it from the moment it was put on..It also eliminates the need for suctioning during the day thus reducing his risk of infection..we remove it at night to sleep and suction him before we put it on. So that's where we are at...

Trachs and EB have presented many problems for us..as if a trach isn't bad enough, throw in EB and we are constantly tackling skin issues...breakdown around the collar, under the neck, and the top of the chest...we found with Casey we have to put a strip of transfer under his collar, slightly larger than the collar to protect him from the edges of the collar...the posey collar seems to be the softest, but the edges still seem to bother him..when his neck is not wounded or broken down, I use a silk fabric to cover the collar and eliminate the need for the transfer..If I use aquaphor or vaseline on his neck, and put the transfer on, I end up with fungal infections from the moisture and the mist collar adds even more moisture to the neck and chest area...so we found the least dressings for casey and air allowing to circulate the area is best..so we try to use the posey collar sewn covered with silk and a thin layer of a lubricant...and nystatin when it's fungal...which is incredibly itchy...I use the mepilex 4X4 with a slit cut under the trach for stablility..I then found that the lotion I have to put on the 4X4 dries up after awhile and then the 4X4 rubs on his chest so I have to put a piece of transfer under the 4X4 on his chest to avoid the rubbing...UGH...we also had trouble with the mist collar strap sliding under the collar and rubbing on his neck while he moved around during sleep so I had to make a fleece "tube" of fabric and feed the elastic through so it would be padded..we had to do the same with the corrugated tubing..I had to make a sleeve of batting and fleece to slip over the tubing to make it softer..he rolls around on it and sleeps on his belly so it was beating up his chest...UGH again...the pulse ox took a bit of figuring out but we were finally able to find one that didn't have adhesive..I found the best way to find things for EB kids are to find out what they use for burn patients and you usually get the perfect thing for EB!!

It hasn't even been a year for us with this trach yet so I have very little to offer here as Ifar as advice as I am still trying to figure it out myself..but I thought I could share his story at least..

Parents should trust their instincts and always demand to be heard...They also need to be aware that this complication does exist in dystrophic EB and we're not sure why more children are not affected by this as this area of the airway is very easily affected by dystrophic EB...Maybe it does go on, but with Casey it happened so many times repeatedly so young that it presented such a problem..Casey baffled and surprised even the most experienced EB professionals in the country with his complications..so just be aware it exists and remember that stridor is NOT NORMAL..no matter what you are being told...and an ENT should be part of every child's EB care and part of every EB clinic. Just routinely...