Ryan is toileting-dependent; we essentially have to do a dressing change every time he needs to go to the bathroom. Fortunately, we have been able to adapt a system that allows for maximized 'ease' during this process. On average, a bathroom dressing change takes about 15-30 minutes, but when he was smaller, they typically lasted up to 45 minutes. Recently, we've had a few 10-minute changes--seems like a breeze comparatively. This DOES NOT include the bath process, which takes about 4 hours daily.
Ryan is trained in using a flask. Since he cannot mobilize at all, he obviously can't get to the bathroom on his own. So, he will tell us that he needs to go. He has alot of fear surrounding using the potty for obvious reasons-his bum is raw, open, and sore. I don't anticipate he will make the potty a regular habit until these wounds are closed. It is difficult to sit him on top of the potty, though, but this problem will be alleviated when he is once again stable in walking and sitting on his own.
Our bathroom routine involves use of a soft polyester fabric to line the diaper and eliminate the possibility of any sharp edges rubbing him wrong. We also cut the elastic out of every diaper and use fleece strips to add extra padding once the diaper is secured.
To clean him, we pat and dab, as opposed to rub. He has very sore areas that haven't ever fully healed after his experience in the NICU, so it remains a problem area.
Ryan's dressings are prepared beforehand, and usually consist of mepilex lite that's used for padding. Occasionally, we will use Vigilon or Magic pads, (hydrogels that promote optimal healing), mepitel, or a product called Contact Layer. Blisters are popped, and Ryan's skin is lubricated with vaseline and topical antibiotics where needed.
We have also found that ointments are superior to creams in the healing process, as they allow the keratinocytes to travel across a wound and heal the skin. Recently we have also included coconut oil in our regimen, and this seems to be helping a bit as well.
We often have to alter our daily 'routine' to match the weather, Ryan's activity level, and the condition of his skin. Most importantly, we know that the heat and humidity increase skin fragility. We have tried to un-wrap Ryan as much as possible in the warm weather to encourage breathing of the skin, but also worry that when he goes to school, he will need to be wrapped at least twice a day (once in the morning to make sure he has appropriate coverage for school, and when he arrives home to ensure there is no damage).
Blisters can grow from the size of a pinhead to the size of a quarter in a matter of an hour, so it is essential that we are adept at finding and popping new blisters. Once we pop the blisters, we use desonide ointment (steroid) plus desitin (zinc oxide) to stop the growth of the blister and dry it up).
We do not typically wrap Ryan's whole body, as we want to allow for maximum mobility, but there are currently a few sore that we 'spot' wrap. These have been incredibly slow to heal! Ryan's nails have fallen off, which is actually a blessing in disguise, as he can no longer scratch himself.
Ryan has had several corneal abrasions and ulcerations with infection--which are commonly seen in EB patients. This impacts his vision. He has used contact lenses in both eyes to help heal and prevent abrasions. In the event he does have an abrasion, we use ophthalmic ointment or antibiotic drops in the affected eye(s) every 30 minutes, and also use ointment throughout the night. Fortunately, application of contact lenses seemed to have helped this issue from frequent recurrence! Ryan also tears quite a bit thanks to a clogged tear duct. Another blessing in disguise, as this seems to provide constant lubrication for him. It may seem like he's crying...but he's not!
Once his dressings are complete, we start our day with a bit of playing and physical therapy strengthening exercises. We do have a physical therapist come to our home once weekly, and we have seen some dramatic results with this.
Ryan loves to go outside, but we are limited (at least in the summer), as he blisters extensively with increased heat and humidity.
Much of Ryan's care is preventative. Without these wraps and lubrication, his skin does not fare as well, and there are more blisters to pop, and wounds to care for. We feel fortunate to have found a system that works for us...at least for now! And, of course, look forward to the day that there is a cure!!!! We are currently using an investigational medication to try and help with wound healing. More to come on that.
Click here for more information from the Stanford Dermatology clinic regarding appropriate care tips for children with EB. For video resources, click here.
You can also reference information on the EB research partnership facebook page or the DebRA website by clicking here.
We bathe Ryan on a daily basis to check his body for new blisters, and to soak his dressings to ensure they do not stick to his wounds. The only baths we have ever missed in his lifetime are during his hospitalizations. Ideally, two people to bathe him, as one is usually holding him while the other scours his body to see what damage was done during the day. Sometimes we add bleach or vinegar to his bath to help prevent infection, although we have been lucky from this standpoint, as we haven't needed to make this a regular part of our routine. We also use chlorhexidine on particularily stubborn-looking wounds. He has been lucky-he hasn't had any skin infections requiring oral antibiotics. Otherwise, we cleanse him with a hypoallergenic baby soap (Cetaphil and Aveeno).
The bath is a painful process as Ryan does have multiple open wounds that affect approximately 40% of his body, especially on his bottom. He requires ketamine and other medications as pretreatment for pain associated with his bath.
These chronic wounds are in part what prevent Ryan from doing 'normal' 12-year-old boy things. For instance, he is extremely hesitant to do anything physical (playing soccer, etc) because he fears an injury. However, we have been very fortunate to become involved in the Challenger League-which safely allows him to participate in baseball!
After the bath is done, we wrap Ryan's legs and torso. He does have some wounds in these areas, especially in the areas where he flexes. .
We generally use a combination of mepilex lite and vaseline on his feet/torso, and tubifast to secure the dressings. We wrap Mepilex Lite over the tips of his toes and around his torso to protect his chronic wounds on his back and side.
We lubricate the rest of his body with a combination of products, but mainly coconut oil in small applications where there are wounds.
We did use aquaphor for some time, and determined that Ryan might be allergic to the lanolin component, as his wounds were just not healing at all. Since we have switched to vaseline (does not contain lanolin), his wounds have started to close! And, it is a lot less expensive, too!
Blisters are popped with a lancet device, and his hair is combed as gently as possible. We then transfer him to his changing table to apply mepilex lite to the wounds on his bottom. Sometimes we also use mepitel in the groin area/areas that flex to prevent sticking of the tubifast components.
The entire process takes about 1.5-2 hours for bath alone, sometimes less on good days. If you add this to time it takes to prepare all of the supplies beforehand, it's no wonder we feel like the bath is a full-time job.
The worst part of the bath is initially getting in. It stings his wounds. We have started to use about a quarter of a cup of salt to help make the water more isotonic. This seems to take some of the 'bite' out of the water.