I first noticed a ridge on the left side of Bella’s skull, above her forehead, when she was a few days old. Our pediatrician said not to worry as most infants’ heads are irregularly shaped. I took Bella back to the pediatrician when she was 10 days old because her breathing was abnormal. Although the pediatrician advised us not to worry about the irregularity when we called over the weekend, I followed my intuition and brought her in the following Tuesday. The doctor didn’t seem concerned and casually said Bella was probably playing with her vocal chords. When I mentioned the term “trachea malatia” (something I had researched over the weekend) she looked surprised that I knew the term and said if she had it, it was very mild.
A few days later we returned due to concern about Bella’s refusal to eat and daily vomiting. Bella was diagnosed with acid reflux and prescribed Zantac. The medicine helped for a few days but her feeding quickly regressed and she stopped gaining weight. I brought Bella back to the pediatrician desperate to find answers and a treatment for her refusal to eat. This was the only doctor appointment her father missed up to this point. The pediatrician looked Bella over and casually said “I’m not concerned about the reflux (mind you she wasn’t eating and only barely gaining weight because I was feeding her every hour or two) but I would like you to see a neurosurgeon.” Without explaining why, she left and a nurse returned to check Bella’s oxygen saturation (because of the coughing and reflux symptoms). The doctor returned and reviewed her notes about putting rice cereal in her bottle and increasing the Zantac. As she finished I asked, “And what about the neurosurgeon?” She said, “Oh yeah, one of our nurses will call you in a month to schedule an appointment with a neurosurgeon who visits once a month.” In shock and struggling to gather my thoughts and maintain my composure, I asked again what it was for. Without explaining craniosynostosis she said the raised ridge (the one I noticed when Bella was a few days old) seemed more irregular and in her opinion should have evened out by then. I left a bit in shock. I called her father once I was in the car and cried at the mention of a neurosurgeon.
Her father, of course, wanted to understand the reason for the referral but I realized I had no idea what it was for other than that her head was irregularly shaped. He called the next day and a different doctor explained the referral was due to the concern that of one of Bella’s cranial plates may be fused. After doing our own research again we learned about craniosynostosis. I wasn’t waiting a month to meet with a doctor who came to the area once a month. My sister obtained the name of a neurosurgeon at UCSF, Dr. Nalin Gupta. I found Dr. Gupta’s email Sunday night on UCSF’s website and sent him an email sure I would never hear from him directly. To my surprise, I had an email in my inbox from Dr. .Gupta 8am Monday morning. I asked him who he recommended to do the initial evaluation and CT scan prior to seeing him should we need to. He asked who told us we needed to see a neurosurgeon and then offered to look at pictures of Bella over email. Excited and nervous I took pictures immediately and emailed them back. Within half an hour Dr. Gupta responded and said he didn’t think her cranial plate was fused but he would “fit us in” when he got back in town next week. We had an appointment one week later. Amazing. We drove from Lake Tahoe to San Francisco the next week.
During the first appointment Dr. Gupta repeated that he did not believe her cranial plate was fused but he would have to see Bella again in two months when she was bigger. We returned two months later afraid he would say she needed a CT scan to confirm whether it was fused or not. After looking at her, Dr. Gupta revised his initial assessment and said he believed it was fused. He explained that his initial hesitation was because the cranial plate that is fused is extremely rare, frontosphenoidal (there are under 10 reported cases of the minor suture alone being fused including Bella). A CT scan was completed 2 days later.
What I forgot to mention was that between the second month and the fourth month appointment, Bella had two upper gastrointestinal exams completed because her feeding continued to be a serious problem. The second one was completed after the gastrointestional specialist reviewed the results from the first one and said it was difficult to be sure from the x-rays but Bella may have a life threatening condition (malrotation) and an artery blocking her esophagus that would require immediate surgery. He told me Bella needed to have another upper gastrointestinal exam immediately. I rushed her over to the hospital afraid for the worst and waited for my husband in the waiting room of radiology. Thankfully the 2nd exam confirmed there was nothing wrong with Bella in regard to the initial concern. Just a terrible scare.
Following the CT scan, we drove back from SF and received a call from Dr. Gupta a few days later. He confirmed, what I already knew, that Bella’s minor suture that ran above her left ear (like a pencil would run if it was tucked behind her ear) was fused. There was a canal the size of a pencil above her ear from the fusion. The surgery was scheduled for 2.5 months from then when she would be 6.5 months old.
All the meanwhile Bella was still not eating well or gaining weight. We were fired without explanation from the first pediatrician via a three sentenced letter even before they called to give us the neurosurgeon referral (thankfully we were already on the search for a neurosurgeon). After calling, we were told it was because I asked about the policy of switching pediatricians within the practice. We obtained a new pediatrician who referred us to the GI specialist for Bella’s feeding issues and repeatedly said “Bella is just petite.” We went to a speech therapist to rule out poor oral motor coordination. She gave us some exercises to do with Bella and then said she did not believe it was a swallowing issue but rather a reflux issue. When no one could help us I reviewed my feeding journals (kept since birth) and realized that Bella had a pattern of eating better at sea level. We packed our bags and left the next day desperate for this to be the case.
I was terrified to have Bella undergo such a serious surgery and be underweight knowing full well she would lose weight and would likely do better weighing more. I took her out of the altitude for two weeks and she ate better starting the minute we arrived. She thrived for two weeks and then stopped eating the day we returned. Exasperated and tired, I called the doctors and they all told me they’d never heard of such a thing. I took her to a friend who studies allergies and uses light treatment to treat allergies. This went terribly – Bella screamed the entire time. Hopeless, we made an appointment with an allergist the next day hoping she had an allergy to something in Tahoe we didn’t know about. The allergist discovered she had minor allergies to a few things including soy beans and a few others that I hadn’t eaten while breastfeeding (I had to pump for 7th months but that’s another story). I didn’t know why but I knew my daughter ate better at sea level so that’s where I was taking her until the surgery. We rented a studio in the Bay Area for the next month. Again her eating improved and she gained weight.
A week before the surgery I called our pediatrician about something else and also asked about a speck of something in Bella’s eye. I asked if it was something I should be concerned about. She said if I couldn’t get it out with saline solution than I should take her to urgent care. Urgent care! What? My heart sank. I wasn’t taking my baby to urgent care a week before skull surgery with a waiting room full of sick kids. Instead I got an appointment with a local pediatrician. Another doctor appointment. The doctor tried unsuccessfully to get the “foreign body” out of her eye. Bella screamed the entire time. The doctor said this was a serious matter that needed immediate attention due to the risk of infection to the eye. She referred us to an ophthalmologist. We resisted taking her to an ophthalmologist because we figured they would have to put her under general anesthesia to get the foreign body out that was partially embedded. We asked the neurosurgeon if a UCSF ophthalmologist could remove the speck during surgery while she was asleep during the cranio surgery. Thankfully we avoided another doctor appointment and another procedure. UCSF arranged for an ophthalmologist to take the speck out at the beginning of surgery.
Bella went in for surgery on June 29, 2011. We arrived at UCSF at 6:30am and waited for surgery until 8:30am. She was in surgery until 1pm. We got to see Bella for the first time around 2pm in the pediatric intensive care unit. Bella stayed in the PICU for three nights because she couldn’t breathe without oxygen. Tim and I spent every night bedside to monitor Bella’s well-being. We tracked every medication and dirty diaper. We did everything we could to insure Bella was receiving attentive care from the doctors and nurses. We met with various doctors and told our story over and over. The UCSF pulmonary doctors were the ones to correctly diagnose Bella’s breathing troubles as laryngomalatia and mild trachea malatia. This goes back to the breathing problems I detected at a week old (trust your intuition). The third day post-surgery was the hardest day of the entire week. Bella’s blow-by (air tube) fell away from her and her heart rate began to crash and she couldn’t breathe. It was terrifying. By the fourth day Bella was transferred to the children’s unit and her bandage was removed. She was stable and needing the oxygen less and less. In preparation to leave the hospital they performed an altitude breathing challenge test to assess her need for oxygen in the altitude. She seemed to do ok on the test but it was unclear whether she would need the oxygen while eating and sleeping in the altitude. They sent us home with oxygen.
Bella’s recovery from cranio surgery was remarkable. In fact, the focus of our concern had been on her eating and breathing. Her scar has healed beautifully and her spirits have remained joyful. She left the hospital with her left eye 80% swollen shut and a huge smile on her face. She never stopped smiling through any of this.
July 29, 2011, exactly one month post op. Bella’s hair has grown back and her left eye was 75% open and less red every day. Her eating was dramatically improved for three weeks following surgery with the oxygen while in the altitude. She ate more than she ever did before getting the oxygen. However, we went back to SF for our follow-up appointments and her eating simultaneously regressed a bit. We met with the plastic surgery, neurosurgery, ophthalmology, and pulmonary teams. Her recovery was considered positive by all teams. However, the ophthalmologist identified a minor lack of alignment in Bella’s eyes (totally unrelated to the foreign body removed from her eye). Of course, it was upsetting to hear of yet another issue our beautiful daughter may be dealing with.
May 26 2012-Nearly 10 months after Bella’s surgery, Bella is doing well. She is now 17-months-old. We have met with more specialists than I can count and we are still left without any answers to her
myriad of medical challenges. Her eating continues to be a challenge. She only weighed 18 pounds when she was 1 years old. We took her to an endocrinologist who, in fact, feared Bella was shrinking. They did a variety of blood tests to rule out the presence of a growth hormone deficiency. According to the endocrinologist Bella is healthy by all accounts according to her blood panel and theorizes that Bella is “a late bloomer.” The doctor believes Bella’s growth was stunted after her cranio surgery and various doctor appointments leaving her without an appetite because she isn’t significantly growing. The doctor believes Bella’s growth will start and hopefully start soon. She isn’t shrinking but her length went from 60% at the time of her cranio surgery slowly down to 20%, 13%, 6%, and now 3%. Her teeth have also been slow to come in. She has two bottom teeth and one breaking through the top.
Bella had eye surgery for strabismus on May 14, 2012 at UCSF. As noted previously, Bella’s eyes were not aligned following the cranio surgery. The surgery was successful and left Bella with what we hope is binocular vision and finally direct eye contact. As we expected, Bella had breathing difficulties during the surgery and required a breathing tube mid-surgery. The anesthesiologist didn’t think she would need it but we were sure she would, given what happened with the cranio surgery. The eye surgery is typically a 2 hour outpatient procedure. Bella was dependent on the oxygen following surgery and required an overnight stay in the hospital (where we stayed for the cranio surgery). I slept bedside to monitor her saturation levels but by morning she no longer needed the supplemental oxygen. We were discharged at 9:30am and went to a follow-up appointment with her eye surgeon. We were allowed to go home with a positive prognosis.
Aside from her eye surgery and eating issues, Bella works with her physical therapist once a week. Bella’s low tone is making it quite difficult to reach developmental milestones such as walking. We are having custom ankle braces made next week to aid her walking. She is cruising everywhere but not standing unassisted yet. Despite her challenges, Bella is resilient and constantly challenging herself to make strides forward.
Her eyes look great and she is happier than ever! Bella is expecting a baby sister in October : ) There was never a family who loved their daughter, granddaughter, niece, or cousin more. Bella is an extraordinary person who I have much to learn by. Her grace and sweet nature astounds me and inspires me to try to be the same. Bella’s story of course doesn’t end here. There will of course be new obstacles and challenges ahead but this is a chapter in her story for us to reflect on and learn by.