Lisa Tack serves on the BCCNS Life Support Network Board of Trustees. As a board member, Lisa helps steer the organization to a sustainable and meaningful future.
What is your connection to BCCNS and when did you first get involved with the Network?
I first learned of the Network in 2009 after my daughter was diagnosed with BCCNS at the age of two years old. My husband and I were seeking information and support, and we found the Network on the internet. We were greeted with open arms from the moment we made our first phone call, and continued to refer back to the Network over the years.
What made you want to get involved on the BCCNS Board of Trustees?
I have a unique point of view on BCCNS due to my medical training as a practicing Dermatology PA-C and as a parent of an affected child. I wanted to be a part of the Board to be able to share my knowledge and ideas as the Network embarks on some amazing new changes. It is also very important to me to advocate for individuals affected with BCCNS, and what better way to do so than to serve on the Board!
What’s something you are looking forward to in 2018?
In 2018 I am looking forward to working with Jean and all of my fellow Board Members to continually make improvements to the Network and to let the world know that we are the leading authority on BCCNS. I am also looking forward to attending regional meetings and getting to know the members of the Network.
The BCCNS Life Support Network and What It Means to Me
by Stephen Douglas, Ripley, West Virginia
When I was diagnosed with Gorlin Syndrome, I was just a kid. I didn’t know what to think. I knew it wasn’t cancer or a big-time sickness. All I knew was I had “it”. For a pretty long time, I was scared. I had never heard of BCCNS, so I didn’t know what it could do to me. What would people think of me having it? Would my friends still want to be my friends and play with me? What would happen to me?
All of these thoughts were going through my mind. My mom had been working overtime finding as much information as she could. One day, she found something that changed our lives, a link to the BCCNS Life Support Network.
It didn’t take long for us to fit in with the group. It didn’t matter if we were at a boxing match or just sharing stories at a restaurant, I felt like I belonged. It’s a feeling you get when you’re with your family. You don’t feel judged; you feel like you can be yourself. That’s what BCCNS is; it’s our bloodline. We’re a family. It doesn’t matter if you’re in America, Canada, or Mexico, if you have BCCNS, then you’re family.
The Network is what brings us together. It’s our link to each other. If you need support, you can count on them to be there. I know I couldn’t have made it without their help. They have provided us places to stay, doctor referrals, lots of information and fun.
So if you ask, “what the BCCNS Life Support Network means to me?” I would tell you it means — family!
I Have Gorlin Syndrome, but It Does Not Have Me
By: Monica Murray
“What’s wrong with your baby?” Mom often heard from the mouths of curious, but ill-mannered children; or from the eyes of curious, but afraid to ask adults. Defensively she would say, “Nothing. What’s wrong with you?” Admittedly, there was something different about me. At the time, we did not know what.
On the 10th of August 1993, I was born caesarean section. My humongous head had been stuck. (I have a big head, both literally and figuratively.) The delivery doctor decided to use a suction cup-like machine to try to help me out. The suction cup was attached on the speech area of my brain. Considering the fact that speech problems come with Gorlin Syndrome, run in the family, and the suction cup debacle, it is a miracle that it does not sound like I am speaking a foreign language. (Honestly, I did have my fair share of difficulty with speech in my early years.)
Placed in the intensive care unit after my tumultuous delivery, I made no friends due to my constant screaming. (My voice is deep to this day… part Gorlin Syndrome accent and part deepened from endless screaming as a baby. A Staples employee once mistook my voice as a male voice over the phone, and a music instructor once thought that I could pass as a male tenor.) To the relief of the babies’ intensive care staff, I went home two weeks later. The hospital staff tried to convince Mom and Dad to take me to see a neurologist. But decided against it for fear that I would turn into an overly drugged baby zombie. (The Walking Dead was not popular yet.)
Over the next few years, my vision gradually decreased, resulting in a visit to an optometrist an hour away. After an eye torture session, I vomited on the way home. Not wanting to repeat history, Mom and Dad took me to the optometrist in the local mall, where she decided that I needed glasses. (I went through torture and vomiting… for glasses?) To add to the frustration, my throat kept getting sore, leading to the first of many surgeries. The family doctor determined that my onslaught of sore throats would require a tonsillectomy and adenoidectomy.
In my medical history, mostly everything occurs more than once, and my adenoids were no exception. The adenoids grew back, leading to another adenoidectomy. In the meantime, my ears repeatedly required surgically implanted tubes. On one occasion, a doctor pulled out a pea-size ball of wax. On the second “successful” time, the hospital staff had to come up with a new operating procedure on the spot just for my ears, which included a second set of tubes. Later, the tubes twisted in my ears, causing me to lose hearing temporarily leading to another torture session, this time for the ears; complete with a vacuum-like object to clean my bloody ears. Needless to say, that was my last round of tubes.
Having a reputation for being uncooperative, I dreaded the possibility of mouth surgery. I tried to be cooperative, but inherited gag reflex, so most of the dental assistants worried that they would be seeing my breakfast in reverse. In the fall of 2004, I started to experience mouth pain and a raisin cookie-like taste in my mouth. Fearing that I had a cavity, I was silent, ridiculously hoping that it would go away. Unfortunately, the pain started to get worse, leading to me falling out of bed in self-defense after dreaming that someone was stabbing my mouth. I kept getting sick, leading to our family doctor declaring that something was wrong. But he did not know what it was. After tearful pleading from Mom, I finally remembered the increasingly agonizing mouth pain. I opened my mouth, and Mom found what she called “a bag of pus.” Mom and Dad talked after I had “gone to sleep,” and they worried that it could be cancer.
The next day, Mom and I went to the oral surgeon who took a panoramic x-ray and could not find anything. Mom saw something that was above my mouth, and she pointed it out. “That’s her molar,” the oral surgeon called out in a panic. “How did it end up there?”
After looking in my mouth and comparing it with the panoramic, the oral surgeon took Mom out to the hallway and asked her if she trusted him. “I think so, why?” Mom questioned in confusion. “This little girl is out of time,” he said. Mom broke down and cried. The oral surgeon asked Mom to pray for him and me. Immediately whisked away, everything became a blur. An IV was in my hand, my blood pressure was continuously tested, and they gave me oxygen. Three hours later, after seeing what looked like blood and guts on a paper towel, my mouth and butt were sore. The oral surgeon had removed the trouble-making keratocyst and molar. He had discovered other keratocysts. To save me from pain, he put tubes in them to drain them with the premise of removing the keratocysts at a future surgery. I later found out that all of the patients in the waiting room had to go home that day because the doctor declared an emergency.
The keratocyst draining tubes turned out to be a nuisance. Dad had to remove a piece of hot dog from one of the tubes in front of everyone at an ice cream shop. They probably lost their appetite after that. Luckily, the next surgery went better.
I was due to have another surgery in December to remove the “last” keratocyst. However, I felt like there was another keratocyst. The oral surgeon could not find it at first, but at the next checkup appointment, he did. He rescheduled the surgery for the day after Thanksgiving. The Black Friday surgery turned out to be noteworthy for more than the name. After the surgery, I felt nauseous. My IV-controlled hand accidentally knocked over the trash can. I tried to call out for help, but a mouth full of gauze prevented me. I threw up a lot of blood… and the gauze. My oral surgeon happened to pass by the room. His panicked, confused, and hilarious facial expression is unforgettable. One of the nurses changed me and, to the relief of everyone, sent me home.
Over the years, I have lost track of how many keratocyst surgeries there have been. (Nine?) After a couple more surgeries, everyone noticed an unusual-looking mole on my forehead. People tried to persuade me to remove it for cosmetic reasons, but I refused because of my experience with a cosmetic doctor (long story short: a practically blind doctor with a laser “stabbed” my thumb wart, just for the wart to grow back). An orthodontist appointment was about to change my mind about the mysterious mole…
My braces were getting removed for the second time. The fact that my mouth required a dentist and two orthodontists for two sets of braces should say a lot about my medical history. During the appointment to remove the braces, the orthodontist took Mom to the side and told her that I was a sweet girl (I think he was talking about the wrong patient) and to take me to a dermatologist before it was too late. Mom cried as she told me this, and she told me that no amount of post-traumatic stress memories of wart doctors would change her mind. Therefore, we found ourselves at the dermatologist office the same day of senior pictures. When the office called us back, the dermatologist told us that he thought that my mysterious mole was a basal cell carcinoma (BCC). In response, Dad folded over as if someone had punched him in the stomach and Mom’s eyes watered. The dermatologist wanted to perform a biopsy that day, to which Mom panicked and told him that I was supposed to take senior pictures.
My senior pictures turned out great, but something else was on Mom’s mind. After my first keratocyst surgery, Melanie had approached her with internet research on a rare condition called Gorlin Syndrome. Mom, frustrated after fruitless fights with the insurance company, angrily told her to stop playing online doctor. During junior year of high school, I was a library aid and used the sources to search for causes of keratocysts. Gorlin Syndrome came up in a medical database. I looked at the condition excitedly, thinking that all of the medical mysteries in my life would finally have answers; however, only one of the criteria matched. Later that day, I told mom about my discovery. Mom told Dad about the possibility of Gorlin Syndrome. He read the information and wept, believing that I did have Gorlin Syndrome. When we went back for the biopsy results, the dermatologist confirmed a Gorlin Syndrome diagnosis.
A biopsy confirmed that the mysterious mole was a BCC. I had Moh’s surgery. Thankfully the dermatologist only had to remove one layer of skin to extract the cancer. In the meantime, my oral surgeon had decided to retire. We found a new oral surgeon through our orthodontist contacts. As excited as the dermatologist was to have a Gorlin Syndrome case, the new oral surgeon readily accepted.
Shortly after, I became a high school graduate. One keratocyst surgery later, I was a college student, living and sweating in the dorms, getting sick every other week. My excess sweat had caused a U.E.O. (unidentified eyelid object) to form on my right eyelid. Medically, it was causing my eyelid to droop and become tired. Therefore, we went to the dermatologist, and he removed it using local anesthesia. My eyelid has a bad history of fluttering. Norman Bates would have been thrilled with the possibility of a knife abruptly meeting my right eye, but the dermatologist and I were not. Therefore, it was a nerve-wracking surgery for both of us, and in the tradition of Gorlin Syndrome, the U.E.O. grew back a couple of months later.
During a post-op visit with my oral surgeon, he noticed the U.E.O. and offered to remove it under general anesthesia. Mom and I jumped at the possibility and he removed it, but it came back. The oral surgeon and my dermatologist referred me to an eye specialist. The eye specialist removed the U.E.O. under both local and general anesthesia, with the diagnosis of hidrocystoma. When I “woke up,” my liveliness rivaled that of a coma patient.
What has perplexed family members the most about Gorlin Syndrome is trying to figure out the family origin. One family member even went so far as to take a notepad along to family reunions and ask family members about mysterious medical ailments. The only weird symptom turned out not to be a part of Gorlin Syndrome at all. What perplexes me the most about Gorlin Syndrome (aside from the keratocyst pain) is when people try to set limitations on me based solely on the fact that I have a medical condition. I am fine with legal and ethical limits; however, I am not fine with limits “based” on Gorlin Syndrome. Someone once told me that I should not try to educate myself or move forward based solely on my medical condition. I responded with, “That is not an accurate reason for me to not move forward with my life. It should not matter if someone has a medical condition or not. As clichéd as it may sound, I have Gorlin Syndrome, but it does not have me.” There is my inspirational thought in this otherwise funny article.
Currently, I apply Coppertone Sport 100+ whenever I prepare to go outside. Mom has me so stockpiled on sunscreen that it could cover a full team of summer volleyball players. So far, I have not had any more BCCs, to the grateful amazement of my dermatologist. However, keratocysts have been in relative abundance. Approximately, I have a keratocyst surgery every six months. As for the hidrocystoma, the eyelid is still recovering as I write this, so we will just have to wait and see. God bless and thanks for reading this article. Special thanks to (in no particular order): Mom, Dad, Melanie and Sean, and other family members that are too numerous to list.
My Journey with Gorlin-Goltz Syndrome
By Margaret Emery
My name is Margaret Emery. I live with my husband Paul and our two sons Damian (who has Gorlin Syndrome) and Simon in Highbury; a suburb of Adelaide, South Australia in Australia. This is our story . . .
In 1989 the oral surgeon, treating my then 14 year old son for cyst and teeth problems, asked if I would take Damian to the Women’s and Children’s Hospital in Adelaide to assist with research. I can vividly remember walking into the professor’s room and him telling ME, “YOU have Gorlin Syndrome”. Hey, wait a minute, I am here for my son. NOT FOR ME. My large head and facial features were clear evidence for him to tell me the news. After extensive tests, x-rays etc. it was confirmed BOTH of us had the condition. What a wonderful 40th birthday present!!
After so many years of wondering, was this the answer to why I missed a lot of my intermediate year; continually healing from cyst problems? To think my parents were first told that an “impacted tooth” was the problem and would need to be removed. Thank goodness they sought a second opinion! What about the times I haemorrhaged and had to have a blood transfusion. After one operation I can remember my father asking me if I wanted to see my face in the mirror. It was so swollen. I could not bring myself to do so. The time my parents were told that I was not expected to live through the night, but I AM STILL HERE!!
About the time of my diagnosis, I had been referred to a dermatologist for a rash on my legs. Yes, they were skin cancers; unusual though to have so many on my legs! Upon confirmation, I remember contacting the doctor to say, “Guess what, I have some news. (No, I am not pregnant!) I have Gorlin Syndrome.” He had previously asked me to find out from my parents if they ever gave me a Bells Tonic, which contained arsenic and could trigger skin cancers. No, they had not, was thankfully their response.
After 23 years of constant appointments with my dermatologist, we have witnessed every kind of treatment possible. Thousands of skin cancers have been removed. Aldara Cream has been tried, at times with moderate success. (At the moment I am using Aldara and it does seem to be working.) Interferon injections have been ineffective for me. I have had considerable Mohs surgeries and would have to say my worst one was about three years ago. There were two areas of skin cancers removed from my scalp which resulted in two large skin grafts being taken from my thigh to cover the affected areas. A plastic surgeon worked with my dermatologist “on this project”! The recovery process took time. From the back I look like Friar Tuck! As hair will not grow back, I had to face another challenge; wearing a wig. It took considerable time to adapt, but am okay with this now.
I am very fortunate to have trust and confidence in my dermatologist. My only gripe is his choice of music! I am thankful to him for only taking Medicare and/or Private Health Fund payments for the length of time I had been going to him. He always looked forward to the challenges I presented! At the end of last year he retired, and I now have another excellent dermatologist.
Having cellulitis on my legs a couple of times resulted in my dermatologist being very wary to do further surgery on them. For the past eleven years I have been having PDT treatment at the Royal Adelaide Hospital, mainly on my legs. Trying to remember from the first treatment to the next as to the areas treated was a real concern in the beginning, but thankfully photos taken now have fixed that problem. Again, no payment is required by me for these treatments.
I also go to an eye specialist, where I have had numerous cysts removed from my eyelids. He continues to check on my eyes.
Also, I have regular Pap Smears and internal Ultrasounds, but thankfully to date I have not had any problems.
A few years ago I had a large cyst on my lower jaw going from just below my ear to the center. My dentist referred me to a specialist, after wondering why this blister type swelling had appeared. What annoyed me was the specialist said it had probably been lying dormant for about five years and had gone undetected. This specialist did “a first” for him by inserting something like a small medical cotton reel in my jaw line. Whereby, with daily syringe washing by me for a period of twelve months, the cyst would shrink small enough for him to then operate and remove. This was successful; better than breaking my jaw! Yearly check ups have revealed the healing process successful and there have been no further jaw cysts.
This year my name was put forward as a candidate for the new drug trial, but after an appointment to check if I had any cancers fitting the required measurements, etc., it was found that I was not eligible to proceed.
As there is supposedly no history of skin cancers or jaw cysts in my family history, I have been classed as the carrier and, as mentioned earlier, passed it on to my son. Both of us have been to a geneticist. She said it is clear we have the condition. It has been suggested that my son have two blood samples collected for the genetic test, to confirm the research findings from 23 years ago. Once this has been confirmed, other family members can have a genetic test to determine if they have or have not inherited the mutation.
I am very fortunate to have a fantastic team of medical people assisting me on my journey with Gorlin Goltz Syndrome. I also cherish my Adelaide circle of Gorlin friends and value their encouragement, inspiration, humour, courage, support and friendship whenever we get together and/or by email. I value the constant and hard work of a dear friend and her husband in Melbourne who got us all together in the first place and continues to keep us well informed through emails and personally on all matters of life, at the same time dealing with her own challenges. My family support is with me on my journey. My gift of faith is precious to me and a daily constant companion.
It was a pity it took so long for me to be diagnosed with Gorlin Goltz Syndrome. As a mother it is not something I wished for my son to inherit. We are trying our hardest to spread awareness of Gorlin Goltz Syndrome to the people we come in contact with. The areas of support are welcoming, friendly and highly recommended.
Always Look for the Bright Side (in the Shade)
By Annewil Stelloo from The Netherlands
My name is Annewil, I am 30 years old and live in The Netherlands. In 2010 at age 27 I learned that I had Basal Cell Carcinomas (BCCs). Two ‘normal’ moles were removed for ‘cosmetic’ reasons according to my doc and turned out to be BCCs. My gut feeling knew all along that there was something wrong. As long as I can remember I have had these pits on my hands, which turned out to be the ‘famous’ palmar pits.
Because of the multiple BCCs and palmar pits I got referred to a university hospital of Rotterdam. One of the first things they told me was that I was too old to be diagnosed with BCCNS. During this process a lot of doctors and trainees were surrounding me. I was feeling like a fairground attraction. They found a few spots which could be BCCs and made a plan for the removal of those spots. Also genetic testing was done, but no mutation was found.
Being told to avoid UV-light and (radioactive) radiation, I had difficulty performing my job as a laboratory technician in a hospital where radioactive assays were done. So I had to tell my team-leader about my condition and a couple of weeks later (August) we had a meeting with the staff advisor. They told me that my contract would be terminated by the end of the year. This was a big shocker, because I was in permanent employment!
The next months were very difficult for me; losing my job and hearing about the genetic disorder I have is life-changing and also the treatments that were planned for the next couple of months. My world existed of applying for a new job, surgical treatments, dealing with the fact that, at that time, our one year old daughter could also have BCCNS and I was referred to the oral surgeon.
In March 2011, I applied for a job as a lab technician for a company which specializes in floriculture. Within two weeks I got hired and started to work there. It is one of the best things that happened. The job is great. I have incredible colleagues. It is a lot of fun. I even developed a new hobby: photography. With the help of two colleagues I got inspired to create nice images! On a good day, with inspiration, the camera is glued to my hands (despite my daughter’s wishes).
Because new BCCs were showing, a surgery for five dental implants was planned and our daughter would begin her annual check-ups (brain-MRI, dermatology, pediatrician). I told the staff advisor of this company about my condition. They accepted me for the things I CAN do, instead of the things I CAN’T do like my former employer did!! That is such a relief!!!
The real downside of BCCNS is that the doctors advised to stop our family planning. We’re very lucky to have our little girl, for now she looks happy and healthy. People around us were asking when she would be getting a little brother or a sister, which was/is very painful at times. It’s not that we don’t want to have another child, but because the 50% chance of transferring BCCNS we decided to stop our family planning. Maybe in the future foster care of something like that can make it a bit easier for us.. But still, it’s tough when people can’t accept/don’t understand our decisions…
The checks I am getting are the quarterly dermatology checks and the annual dental implants check.
The last derm visit was April 22nd and the doc now trusts my gut feeling (because I’ve never been wrong before, concerning the BCCs). Four biopsies were taken, the only thing I want to know which treatment they will plan for me and when.
In a way, I am grateful for the things Basal Cell Carcinoma Nevus Syndrome (BCCNS) has brought me: a job which I love, a new hobby and an opportunity for self-development. Beside that, a large family on Facebook. J The sentence: “Consider yourself hugged!’ means the world to me. Thank you all!!
Being the spouse of someone with BCCNS (or any other condition for that matter) can be a challenge. I do not mean to imply for a single second that I regret having Christen as my wife. I don’t. She was chosen by God for me, and I am grateful. She is the best decision I ever made. What I mean is that her having BCCNS spotlights my own shortcomings. I freely admit that I am terrible at recognizing that sometimes, she just wants me to listen. To trulylisten. And to only listen. It is at those times that I realize my real role is to sometimes just support her. Our journey over the past four years has taught me that.
That lesson was crystallized for me on three occasions. The first was when Christen came home from the dermatologist with the news that she no longer suffered from “various unrelated symptoms”, but that there was indeed now a name for it. The second was when she returned from the BCCNS Life Support Network colloquium in St. Louis after having met so many others who shared her plight. And the third was when she wrestled with the notion of whether or not we should have children. It is one thing to bear your own cross, but it’s another thing altogether to cast those same burdens onto those you love.
We researched our options and were delighted to discover that, thanks to recent advances in modern medicine through in vitro fertilization, doctors could safely screen our embryos and identify the genetic marker which indicated a positive finding of BCCNS. Only embryos that were negative for BCCNS would be implanted and we were “guaranteed” a child free from the syndrome.
But, the best laid schemes of mice and men often go awry.
For those of you who don’t have the luxury of knowing my wife, the best one-word description I can give you is “prepared”. No matter what life throws at her, she is fully capable of knocking it out of the park because, somehow for her, life has a way of tipping its pitches — to borrow a baseball metaphor. There simply are no surprises. But, not this time. She was unsettled. Oh, the cribs were assembled. The nursery was done. A year’s supply of matching outfits were hung neatly in the closet. “What to Expect When You’re Expecting” had been read. She was physically ready, but I don’t think she was mentally ready. Not quite yet. And looking back on it, I think I now know why. Everything had been too perfect up to that point – and she was sensing the curve ball.
I was blissfully ignorant.
Christen’s water broke, ironically enough, at 4:00 a.m. on Mother’s Day, May 11, 2008, some twelve weeks prematurely. It is hard to put into words the emotions we felt that morning as we drove the sixty miles to the hospital. “Shock” doesn’t do it justice. “Terror” is probably closer to being the appropriate adjective. She had been to the doctor only five days earlier and everything was normal. We were having twin boys and I think I can speak for Chirsten when I say that we were, in our own ways that morning, processing what was happening and silently praying for their safety.
I recall looking at her that morning as we pulled into the hospital. She had never looked more beautiful to me.
Christen and I lived six hours from family, or as we like to say, “Just fifteen minutes by telephone.” And while it is somewhat ironic that the boys were born on Mother’s Day, the practical effect of that irony is the rest of our families already had separate plans. So, we told them all to enjoy the day together, that the hospital had given her medication to delay delivery, and we spent the day (between contractions) watching the final round of the TPC Championship from a hospital bed – alone together.
Her OBGYN finally came to see us at almost 8:00 p.m. After taking one look at Christen, he told the nurses, “She’s going into labor. Dad, put on these scrubs and meet us at the Delivery Room.”
Do what? No, you said we weren’t going to have them for a few days. You said everything was going to be okay. We told our families to stay away. My mother, and mother-in-law alike, are going to skin me alive for making them miss this moment.
Damn. Curve ball.
The next half hour is a blur for me. It was for Christen as well, but at least she had the luxury of being medicated. William Robert weighed in at 3 lbs, 6 oz and Patrick Reagan was 2 lbs, 12 oz.
They were the second and third most beautiful things in the delivery room at that moment.
Over the next several days, we were swarmed with visits from friends and family alike. But Christen sensed that something was wrong with William. His head was entirely too large for his little body. Macrocephaly is a symptom of BCCNS. And so is hydrocephalus. William had both. You can imagine the combination of excitement and dread that we both shared.
Damn. Curve ball.
For the next three months, we made the commute daily (sometimes twice) to and from the hospital to see them. We were able to have our sons all to ourselves, completely untethered from any nurses or doctors, for six joy-filled hours on Father’s Day.
At some point, a blood sample was drawn from William and sent to a different lab who confirmed that he did, indeed, have BCCNS.
Damn. Curve ball.
Suddenly the doctor who had screened the embryos and his lab were no longer accessible. Phone calls and e-mails went unanswered. But there was no time for search parties. It was becoming clear that William’s head was developing abnormally. Doctors confirmed that he had sagittal craniosynostosis. A suture at the top of his skill had fused together prematurely and was causing his little head to become more and more elongated with each passing day.
Damn. Curve ball.
Meanwhile, Patrick was meeting one developmental milestone after another. For him, it almost seemed too easy. He was our “Champ” – a moniker that he still carries. Our hearts were torn. We had one child who was thriving, almost without any effort whatsoever, and one who never stopped crying because the intracranial pressure build-up meant he had a headache every second of every day. And we were scheduling one doctor’s appointment after the next. Thank God for family and friends.
An incredible team of surgeons at Children’s Medical Center of Dallas removed a 17 cm strip of bone from the top of William’s skull once he got to five pounds and was “big enough” to withstand the anesthesia. For months, Christen or I flew with William to Dallas to be seen by his doctors and to make certain his little helmet (adorned with the requisite Texas Aggies stickers) fit properly. At some point, the travels became too much to bear, so our family was separated while she lived with her parents more closely to the hospital. My only connection with my wife and family came via pictures that were e-mailed to me and phone calls where I tried to memorize the sound of their voices.
After William’s suture healed, he was ready for his VP shunt which would relieve the pressure and ease his crying. The moment he woke up from that surgery, he was an entirely different baby. No longer did his plaintiff whaling reverberate throughout our home. He was immediately transformed into the most loving child imaginable.
Our family circle was complete.
Follow-up visits with the doctors came with a litany of restrictions. He will never be able to play football. Or box. He is supposed to avoid bounce houses or any other activity where he could strike his head. Caps and sunscreen are as much a part of his daily attire as underwear, but for him it’s normal. So what?
I went from being the spouse of someone with BCCNS to also being the father of someone with it. Again, so what? I am the most blessed husband in the world. I watched the woman I love (in the midst of dealing with insufferable guilt – though you will never get her to openly admit it) plan, organize and host a 5K race in our home town that raised thousands of dollars for the BCCNS Life Support Network as a way of giving back to those who had helped us when we needed it most.
If you are considering whether or not you should have children due to the increased risk of transmitting BCCNS, we would only recommend that you pray on it. God gives each of us what we can handle. We support one another with whatever burdens they may bear. That’s what being a family is about.
A curve ball is no different than any other pitch. If it comes across your plate, trust in yourself and swing for the fences. Christen has taught me that. Blissful ignorance only gets you so far.