Establishing your child or self with an EB Clinic

                                                  

I have seen a lot of requests for information about EB clinic visits.  What is an EB clinic?  How would going to one help me or my child's care?  What do they have to offer that my regular doctor doesn't?  I am going to detail the benefits of being involved with an EB clinic and will also re post the list of EB clinics that I have gathered, and featured in my first post of this blog : )

                                 Your EB Center Clinic Visit

When you go to an EB clinic it can seem overwhelming because you don't just see one or two specialists...you see them ALL.  By that I mean every main physician in every aspect of EB care.  Gastroenterology, dermatology, surgery, OT, PT, Psychological, Social Work, Orthopedics, Gynecology, ENT, Ophthalmology, Cardiology, Pain Management, Dental.  They cover all the bases.  Some of you may not need to see all of the physicians, for example I don't need to see OT or PT as I don't have issues in those areas, but any area that you have concerns about will be addressed at your appointment by the appropriate department.

A general clinic day goes a little bit like this: (I am using my schedule for my next appointment to give you an idea of what a typical clinic day is)

10:15 am Endocrine Dept. for Dexa scan (bone density) time involved: 15 min

11:00 am  Cardiology Dept. for an ECHO time involved: 90 min

1:00 pm  EB Center Multi Practice Clinic team consult time involved: 2-4 hrs

It is during the team consult that you see the majority of the doctors.  If you have an ENT (Ear, Nose and Throat) appointment or a dental appointment they will be scheduled separately and you would go to those departments for the appointment.

                                    

                           Dental Visit

In regards to Dental visits I just have to say the dental team is AWESOME!  The technology that has been created in the last 5-10 years for dental care for EB patients is amazing!  For those who have had to deal with dentists who are NOT familiar with EB, see if this short conversation sounds familiar.  

Imagine you're at a regular dental office for a cleaning or x rays and you have EB...

Technician: "OK, i need you to open your mouth wide"

Patient: "OK"

Technician: "No, open as wide as you can"

Patient: "I am"

Technician: "Can you just open your mouth a little bit wider?"

Patient: "No"

Technician: "Why?" 

Patient: "As I explained before you began your examination I have EB.  A rare, genetic connective tissue disorder that causes severe blistering and scarring in my mouth and other areas.  My tongue is completely fused to the base of my mouth and has no mobility.  I don't have a gum line as my gums and inner lip/cheek has fused together, which means you won't be able to use a regular x-ray card, you'll have to do a panarex.  Also, I can only open my mouth so wide because of scar tissue that is causing strictures at the corners of my mouth"

Technician: "Are you sure you can't open just a little wider??"

Patient: exasperated, yet polite "No"

Technician: "OK, hold on, I'll be right back"

5 minutes later a new technician walks in and says

"What seems to be the problem?  The other tech said you can't open your mouth? Is there a reason why you can't open our mouth?

OMG!

New Tech: "OK then, let's see if we can try and get an x-ray"

Patient: "OK, but you have to use the pediatric cards as the adult card won't fit"

New Tech: "Are you sure?" 

Patient: still polite ; ) "Yes"

Technician: "Maybe you could just try"

Patient: "No, if I try the adult size it will cause blisters in my  mouth and they're very painful"

New Tech: "I'm pretty good at getting these to fit, are you sure you don't want to try, just once?"

Patient: "No, they won't fit no matter how you bend them or try and place them they wont fit.  If you want an X-ray you have to use the small cards, and even those have to be bent.  Also, you wont see anything below the gum line as the area is fused so the only way you will get an accurate view of all of my teeth is with a panarex"

New Tech: "How about we try the small card and see if we can't make it work..."

And so it goes until they realize you're right but not before they have made you cry by trying to force these plastic x ray cards into your mouth b/c they think you're faking it b/c they've never heard of EB.  At which point they take you down the hall to do the panarex.

Your dental appointment at an EB Center goes something like this:

Technician: "Hi, how are you? M name is sue.  Go ahead and sit down and we're going to get some x rays."

Patient: "Um, OK, but they don't fit."

Technician: smiling : ) "I'll be right back" 

She comes right back and hands me a small plastic card (about 2x3 inches) and says "OK, just hold this on the OUTSIDE of your cheek and be still"

Patient: "Um, OK"

Technician: "Hold your breath while I take the picture...OK, you can breathe" and she takes the card away and we repeat the process on the other side...and your done : )

The x ray cards are very flat and are positioned on the outside of your cheek, along the jaw line for upper and lower teeth.

I was so amazed I was almost in tears at how well the appointment went.  I didn't have to EXPLAIN ANYTHING to them.  Nothing.  It was amazing.  They have special tooth brushes that are made to fit the grip of a hand that is fused or contracted.  After the x-rays you see the Dr and go over your information and a treatment plan is formed for all your dental work.  Now, if you're an adult they will do the x-rays and the consult BUT you do have to be referred out to an adult dentist or oral surgeon but they will help find one for you that is familiar with EB.  Normally they refer you to dentists or oral surgeons in the area of the EB center as those are the ones they know, BUT they will happily consult with your regular dentist or try and help find a dentist or oral surgeon where you live that will agree to consult with them, if your dentist won't.

On to the clinic visit : )  It is a long day, so be prepared with snacks, drinks and entertainment for yourself or your child.  I do believe the children get welcome bags that come with a few things for entertainment and I believe they still give hand made blankets to new patients.  Even the adults get a blanket at their first visit.  My blanket is Tye dye fleece and I love it : )

At this point it is a parade of doctors and nurses and examinations and questions and it can seem overwhelming BUT it is so worth it!  Once the doctors are done you are free to go.  At the end of the day the doctors have a conference where they discuss the patients and begin working on each patients individual treatment plan regarding the issues that you discussed with each department during your appointment.  If your current doctor is willing to work with the EB center doctors then the EB Team will consult with your regular doctor.  If your doctor will not consult with the EB team,and if you don't live close to your EB center, then the nurse educator will help you find a doctor in your area or at least fairly close by that is familiar with EB and would be willing to consult with the EB team.  In my experience they do everything they can to make our lives better regarding medical care.

They introduce you to medications, ointment's, creams, bandages and other products that you may not know about which could help you or your child heal better, faster and with less pain and infection.  Before I went to my first clinic appointment I had 8 major non healing wound areas.  I had been asking my dermatologist for YEARS was there anything new that I could use regarding ointments, bandages and he always said no, nothing...keep using bacitracin.  When I left that clinic appointment I had Medihoney, Alwyn Cream and Bactroban as well as countless bandages to try.  Six months later, using Medihoney, Alwyn Cream and Mepilex brand bandages I went from 8 major non healing wounds to 2 as a direct result of their care and the products they told me about.  My quality of life improved 100% after my clinic visit!

I still only have 2 major non healing wounds.  The other areas still blister and I do have issues with those areas but NOTHING like before my appointment.  They arm you with the latest wound care information and products that are on the market, and give you the information to wound care supply companies who, in turn, bill your insurance for you (and often fight your insurance for you!) and then mail your supplies to your house.  

I use National Rehab and Bill Cornman is the EB rep and he is awesome.  He will get your bandages to you within a few days of your order.  Also, it is their company that produces the newsletter "The EB Advocate".  Danielle Malchano is their insurance Guru and she is amazing : )  There are other wound care companies but I have only ever worked with National Rehab and I really like them.  Their customer service is amazing and everyone you speak to is nice and helpful.

OK, to address the questions I mentioned above

1) Would it be better to wait until my child is older to go to a clinic appointment?

-I believe that it is better to be established with an EB center as soon as possible.  They offer so much information and support and can only HELP you with your child's care.  The earlier you establish that connection the earlier you will be able to connect with the doctors in your area that may be a better fit for your child's care than the one you currently have, or if your current doctor is amazing but knows nothing about EB the EB center team will consult with that doctor so if he/she has any questions or concerns you will still be getting help from the clinic team between visits.  BUT, you will only get that extra help if you have been seen by the clinic doctors, because they can't treat a patient they've never seen.

2)  I can't afford the travel expenses to go to a clinic appointment!

-  There are organization's that can help you get to your appointment.  One of those is The Butterfly Fund.  Their web address is thebutterflyfund.org  Make sure you use this address as there is another butterfly fund but it is not the one that helps EB.  You can ask the clinic coordinator or the nurse educator of whatever EB Center you are going to for possible information on local organizations that can help with travel expenses.  Also, contact your health insurance.  They may be able to help with travel expenses.  I know there are some insurance companies that do this, but I don't know which ones.

Most large hospitals have Guest Services and they have information on local hotels, who often offer discounted rates for those being seen at the hospital, shuttles to and from airports and hotels, free passes to local entertainment (Zoo, etc...) information on local restaurant's, and other information.

There is, generally, a Ronald McDonald House by most Children's Hospital's.  The Ronald McDonald Houses (RMH) are amazing.  You never hear about them or how much they help people.  Their general nightly rate is $25 (it may be different in other states but it shouldn't be much more than that) and if you can't pay that much then you pay as much as you can, even if it's only $5 a night.  If you can't afford that, you fill out a financial aid form and if you qualify (and you probably will) you pay nothing.  

They have a large kitchen/dining area that has, within it, 4-6 full kitchen areas (counter, dishwasher, stove, oven, microwave, sink, dishes, utensils etc...) that you can you use for cooking your own meals.  You get your own pantry, refrigerator and freezer space for your groceries, however, there are volunteers that cook dinner every day of the week (included in your stay) and often volunteers that cook lunch as well.  They have a pantry with dry and canned foods that you can use and cook for yourself or if you want to make a special treat for the other guests.  You are just asked to clean up after yourself.  They also supply dairy products (Milk, eggs, yogurt, butter, cheese) and basic staples like bread, peanut butter, jelly, condiments and applesauce and juices.  There are always leftovers from a previous meal and you are encouraged to eat leftovers so they don't have to be thrown out, but you don't have to.  I bought groceries but honestly, I never used any of them during my stay.  I didn't need to.  

They also have laundry facilities, multiple entertainment areas with TV, VCR/DVD and a movie selection you can borrow movies from.  Their is a PC and Internet station, library and craft room.  They offer free tickets to local attractions (Zoo's, Aquariums etc...) and also have outdoor play areas.  The only thing is that rooms are on a first come first serve basis.  You call the RMH 24 hours before your appointment and they put you on the waiting list.  Normally, by the time you arrive or later that day they may have a room for you.  There is a possibility you might have to stay a night in a hotel while you are waiting for a room to open up, but they will get you in as soon as possible.  I only had to stay one night in a hotel, but I know many others who didn't have to.  It just depends on how full they are.  There is no time limit for your stay.  As long as you or your child is either in the hospital or being treated out patient by the hospital then you can stay as long as you need to.

3) What else does being established with an EB center offer for me or my child's medical care?

-EB Center's have the most up to date technology for Anesthesiology.  Since airway issues are so prevalent in those with EB intubation can be a tricky thing.  The anaesthesiologists at hospital's with EB center's are well versed in the care of an EB patient.  They are extremely careful with every thing they do and take special precautions to reduce the potential for complications.  In short, they are amazing!  I am 38 and have NEVER been intubated before.  I recently had oral surgery and I was terrified because I was going to have to be intubated.  They intubated through my nostril and used a fiber optic scope to guide the tube and there were no problems at all.  Besides some minor throat irritation I couldn't tell I had been intubated.  There was no blistering, no trauma, just very minor throat irritation.

-If you need to speak to one of the team doctors you can contact the clinic coordinator and that person will either find the answer you need or have one of the EB clinic Dr's call you.  It is very reassuring to know that if I am experiencing something new with EB I can take a picture of the area on my phone, then email that pic from my phone to the clinic coordinator or the nurse educator and they forward it to the Dr and within a day, two at the most, you have information about what may be going on, whether you need treatment or if what is happening is normal.  If you need treatment they can coordinate with your GP to resolve the issue, or they may suggest you come to them if it's a serious issue they feel they should treat.

That is my experience with an EB center Clinic.  In June of 2010 I moved from Florida to the Cincinnati area so I could be near the doctors at CCHMC (Cincinnati Children's Hospital and Medical Center).  Clearly this is not an option for many people, and it happened at a point in my life that it was time for me to move from the situation I was in and into the next part of my life.  I am thrilled with my move but I have been in the position where I was driving or flying to other states for proper medical care as the doctor's that were in my hometown simply did not have the experience to properly treat me. 

When I was a kid I spent 3 months every summer at Chapel Hill Hospital in Chapel Hill, NC.  Dr. Brigamon and Dr. Jo David Fine were my dermatologist's, and Dr. Timothy Wright was my dentist.  I don't know how many of you recognize those names but they are some of the key doctor's that spent a major portion of their career's doing research for EB, and are still active within the EB community.  At that time Chapel Hill was one of the only EB center's in the states.  I went every summer for 8 years.  I was poked and prodded.  They took blood and biopsies.  I had more dilatation's than I can count.  All of the doctors and nurses knew me by name.  It was like a second home to me.  This was in the '70's when they still knew so little about EB and I was a curious and confusing patient for them.  Biopsies confirmed RDEB but I wasn't presenting as a typical RDEB patient.  I had all of the internal difficulties and about half of the external ones.  There weren't any tests for sub typing then so they didn't know that I had a sub type that made RDEB less severe for me in some aspects.  

Doctor's WANTED to learn about EB and I was eager to help.  As I grew up that stopped being the case.  New doctor's were less and less interested in EB and how it affected me and eventually they didn't care at all.  They hadn't heard of EB, or if they had it was only a minor mention from med school and no real information.  They couldn't pronounce it, much less spell it and as far as they were concerned it wasn't a big deal.  My mom taught me to fight for myself by always advocating for me.  She made sure I had the BEST doctor's and the BEST medical care she could find.  She sacrificed everything to care for me and I am eternally grateful to her for her love and dedication.  It was from her that I learned how to find the best doctor's as an adult and to not allow any doctor to make me feel unimportant.  To not allow ANY doctor to dismiss me because of their own arrogance and ignorance and I didn't.  I learned so much from the doctor's at Chapel Hill and I used that knowledge to inform the doctor's I had as an adult...and they mocked me for it and that is when I began having so many issues with doctor's refusing to accept EB and refusing to speak to the EB specialists.  

I do understand the fear, frustration, anxiety and anger that one can experience when dealing with a doctor that refuses to listen or believe you when you try and talk to them about EB.  I have been to doctor's that were demeaning, arrogant, rude, disrespectful and down right nasty.  I've been to doctor's that accused me of lying about having EB, or simply did not believe me when I explained the difficulties I have swallowing as the result of an extremely narrow esophagus. That is why I am writing this.  It is up to you, as parent's, to advocate for your children so when THEY grow up they can advocate for themselves and establishing a good rapport with EB specialist's when they are young will only help them as they grow.  They will always have that connection to an EB center and will always have specialists that listen to them and KNOW how to care for them.  EB center's are an invaluable resource and I would encourage every patient/parent to think about establishing themselves with an EB center near them.  I believe it can only help create a better life for anyone with EB.

Here are some opinions from parent's/patients that have been to an EB clinic : )

"We go to cinci yearly it is great because you can ask questions anytime. We get to see all the docs at once in one day. They educate there staff so when we are there for procedures they know about EB all over the hospital.we use the nutritionist freq. we can call there pt or ot for any questions. We like all the docs and the nurses are great. The only bad part is its in Ohio were in NY."-J.N.

"I don't see any cons to it at all. I found it to be the most pleasant doctors visits that we have ever had. It was really nice to go to people that knew more about EB than me. The only thing I wish they would offer is some sort of get together on clinic days. Or at least a contact sheet that people can fill out and view if they want to meet with other EB families while there. When we brought Jackson there he was young and we had not met anyone else with EB. It would have been nice to connect with another family."-M.W.

"i like how he gets to see all the important docs, including social service and community suggestions. i like that it is centered around what is going on in our lives. they take the time to listen to us and listen to my ideas, concerns and issues. i also like that they have a music therapist come in and make Liam smile while all these weirdos in white coats stare at him and take pics! we go to a clinic in NYC @ Columbia pres"-M.C.

"I LOVE being able to live pretty close to an EB clinic!
-MY FAVORITE THINGS-
1. The doctors know SO much about EB (Way more than other hospitals). 2. You don't have to make a bunch of different appointments to see all the different doctors, you get to see them all in one appt. 3. You get to meet a lot of new friends with EB, kids and adults!
-LEAST FAVORITE THINGS-
1.Being able to see all the doctors in one appointment is good, but the bad thing about it is that your there FOREVER! Your there for about 5 hours! (Just a tip...bring SNACKS!)"-N.S.

Below is a list of some of the EB center's.

Cincinnati Children’s Hospital Medical Center, Cincinnati, OH

Contact Donna Engells

Clinic Coordinator (513) 636-2009

Stanford School of Medicine, Palo Alto, CA

Contact Loraine Spaulding

Clinic Coordinator (650) 804-4820

The Hospital for Sick Children

Toronto, Canada

(416) 813-1500- ask for the EB Clinic

University of Massachusetts
UMMHC, Hahnemann Campus
281 Lincoln Street
Worcester, MA 01605
Phone: 508-334-5979 - ask for EB Clinic

Herbert Irving Center for Dermatology and Skin Cancer

Herbert Irving Pavilion, 12th Floor
161 Fort Washington Avenue, New York, NY

Columbia University Dermatology Associates

16 East 60th Street, Suite 300
New York, NY 10022

To make an appointment at either location call 212-305-5293.

Phoenix Children’s Hospital

1919 E. Thomas Road, Phoenix, AZ 85016

(602) 933-2053

The Children’s Hospital Aurora

13123 East 16th Avenue

Aurora, CO 80045

720-777-8445

Sydney Children's Hospital

Sydney, Australia

Contact # 9382-1470 (not sure what the area or country code is)

There are other clinic locations.  To find one near you contact DebRA.org