Leo* Generous, Faithful, Caring, Original, Outgoing
*JILL’S ASTROLOGICAL SIGN; MESSAGE ON A FAVORITE T-SHIRT
Life is a game that can be hard. It’s a roller coaster ride. Both the good and bad memories stay with you. You will laugh and cry, learn and sometimes get burned, love and hate and that’s what I had to learn throughout the years. Life is not fair. A regular day for me can be getting up first thing in the morning, having to worry about whether or not I will make it through the day without an attack, what will the kids be like and staying focused on the school work and not the drama. Unfortunately something always happens to me. My mom says that for some reason, trouble just seems to find me. I don’t know why.” – Jill
Sadly, too many children carry heavy crosses and to many adults respond to such burdens with talk of childhood resiliency, “He’s/she’s young—he/she will get over it” and if “character building” is not said, is implied. The theory is routinely intoned by many parents, psychiatrists, psychologists, medical doctors, educational professionals and others. There is no doubt some children often can and do rebound after setbacks. But setbacks are one thing; crosses and lifelong burdens another. Being humsn, children do have resiliency–but also fragility. And while children are often resilient, as humans we are also residual beings. Those physical, emotional, medical and/or social crosses can surreptitiously reappear in the future, impacting individuals and society, sometimes for better, sometimes for worse.
Manifest acute intermittent porphyria has been Jill’s cross to bear since sixth grade. For her, it is not a static disorder. MAIP gouged a gaping hole in her tween and teen childhood years and pretty much took over our family’s life as we struggled to help her cope with its various forces. She lost the better part of three full school years then. She was then shunted to schools for troubled students largely because the school district used the SE-OHI designation with malevolent intent so it would not have to deal with the bullying issue and the problematic school IAQ/Jill connection. What I had hoped would be her educational salvation turned out to be anything but.
With society’s “expected child resiliency,” Jill dealt outwardly with a gargantuan medical condition as nothing more than an inconvenience to everything she hoped to achieve in life. Only once do I remember her initiating a conversation about why she “got” AIP. It was during eighth grade and she had left school early again; we were on our way to another heme treatment. Mrs. Podurski told me that Jill had become teary-eyed while preparing her backpack to leave. As we approached NHS, she softly murmured, “Why do you suppose I got AIP?”
I answered, “A lot of kids get rare diseases—no one ever expects to get that kind of news.” She replied passionately, “Well, no one should have to get this disease. No one. I don’t care if they are twenty, thirty or sixty years old. No one should have to live with the pain and scariness that AIP makes. No one.”
Sometimes I’ll be in my room wondering, why me? Every now and then my disease will get to me and I don’t understand. Other times I’ll be in school and start to feel side effects from the medicine or symptoms of the AIP and I just break down. But I’ve also realized that I am who I am and this is what God wanted for me. I’ve learned to cope with it as best I can and always try to rise above the times when I wish I didn’t have AIP because in the end I know that’s not going to happen. So many people have said to me that middle school is one of the hardest times to get through and when you live in a small town it can be harder to deal with the drama. Sometimes the drama will cause me to have an attack because I get so overwhelmed. Every morning so many questions run through my head and I just need to know that everything will be okay, but see I am an extrovert and tend to open my mouth way too much. I get caught up in the moment and don’t care who you are. If you’re giving me trouble, I will stand up for what I believe in. And unfortunately because of that I am labeled a troublemaker. But a lot of people don’t understand what I’ve been through and so they don’t know what to think or just don’t care so they give drama. — Jill
It helped considerably that Jill’s MAIP activity calmed down once she entered Burton Academy, allowing her to keep porphyria from the forefront for months at a time. But the ridicule and scorn from LMS peers and adults had made indelible impressions on her psyche and feels of fear and depression often came back, especially when actions and words from B.A. students were directed at her.
I happened upon a stack of papers in Jill’s bedroom, with a title page of “My Name is Jill Gould. I am a survivor of bullying.” When I asked about them, she said they had been part of a planned script for a YouTube message “to help victims of bullying.” It was clear the school bullying she had experienced had been more to process for her than AIP.
During her lengthy latent porphyria period, Jill enjoyed a break from heme treatments. The notion that heme could be considered a preventative measure as far as exposure to certain toxins was concerned kept niggling at me. After everything Jilll had been through, I did not necessarily think that was so. Then, almost to prove it, a sequence of events beginning in late fall 2012 launched a round of MAIP activity and ushered in a return of Purple Days.
An odorous smell was in Jill’s bedroom one morning. She felt “yucky” but went to school where she promptly collapsed and was transferred to NCH’s ED. Even after infusion of the first round of D10 followed by heme, Jill’s head, back, leg and abdominal pain and mental status changes escalated. In addition to mounting pain, she complained about being blurry vision, dizziness and an icy pain shooting thru her hands to her fingertips, “My hands feel like they’re going to fall off!” She insisted I stop shaking her bed. I was standing a foot away from it. She begged for morphine which she had always tried to forego because, “I hate how it makes my head feel!” She was having difficulty breathing and asked for oxygen. She said, “My life sucks, Mom.” She asked if she was going to die.
Although I firmly answered “No,” I was nervous. The ED doctor and I agreed to stay with the protocol and go ahead with the heme then the post-hydration D10 infusion. Having just read the Silent Spring chapter concerning cell respiration, I asked him if he would increase the oxygen level while infusing heme. He did. Within twenty minutes Jill “came back,” brightened considerably and wanted to go home.
Her new beau, Aaron, and his family members were heavy cigarette smokers. Jill often came home from visits there feeling nauseous, achy and “my nose is stuffy—maybe I’m coming down with something.” One day their home furnace malfunctioned and filled the house with fumes. She called to be picked up. We immediately opened all the car windows, even though it was January with below freezing temperature, for fresh air. She went directly to bed when we arrived home. The next day she complained of nausea, headache, aches and pains. She did not go to school. By the next morning abdominal and back pains came screeching in and she ended up in the ED for a heme treatment. It took longer than usual for her to recover following that treatment, too. A few days after that incident, the B.A, school nurse called to say that a staff person had been spray painting in a storage area and indicated that Jill was not feeling well because of the paint fumes. She asked permission to give Jill her inhaler. I suggested she take Jill outside then give her the inhalers so she could breathe clean, fresh air. I said I would be right there to pick her up and asked the nurse not to allow her back inside until the building had been fully aired out.
Thankfully, B.A. staff members remained vigilant about keeping identified toxins such as black dry-erase markers, which they had banned, away from Jill. Also, they did not use the same cleaning products as Littleton school district did. The, in mid-March, 2013, Jill was ill for more than a week with an unusually bad cold and a heavy menstrual period combination. Following that, she remained fatigued, irritated and anxious. She could not sleep, grew nauseous, had aches and pains and complained on and off about headaches. After attempting a few days of home remedies such as rest, plenty of fluid intake and eating a high-carb, multi-grain diet with simple sugar snacks in between, I arranged for heme treatment. However, before we would get to the appointment Jill begged to be taken immediately to the ED. Asked to describe her pain, she rated it eight on a scale of one to ten. She said it felt like her back, leg and stomach were on fire and asked for morphine that hospital staff quickly administered. By the time heme arrived from the hospital pharmacy, the pain level in her back had decreased only to a “two or three.” Then, midway through the heme infusion, her back pain ramped up to a “seven.” The doctor ordered more morphine. In a few minutes, the abdominal and leg pain disappeared but the back pain leveled off only at “two” which lasted until about one-third of the way through the post-hydration glucose infusion. By the end of the infusion, Jill complained that her back pain was up to about a seven again and reiterated her “My life really sucks, Mom!” refrain. But the infusion was finished—and accordingly, so as the emergency. It was time to go and hope the heme would work. We had an appointment to see Dr. O’Connor in less than two days, and we could always call him if the pain did not relent. As Jill and I drove home, I tried to think what he genesis of this recent attack could have been. Suddenly, an illustration from a favorite AIP article (http://www.artcim/products/p14441601075-sa-i6715325/nucleus-medical-art-illustration-of-red-blood-cells-rbcs-transporting-oxygen-molecules-bonded-with-hemogloben.htm] showing hemoglobin transporting oxygen flashed into my head. A head bell chimed and I had a forehead-smacking thought, “Oxygen! Problematic heme/oxygen binding is involved with MAIP activity. We need to add oxygen during the heme treatments! Oxygen had worked well in January—and it had worked from day one back in 2007. In fact, she had improved every time supplemental oxygen had been administered or fresh air brought in when she was in AIP distress. WHY hadn’t I thought of it before?” I said to Jill, I just thought of something that might help you next time. Remember in January when you had that attack and I asked for oxygen and you got better within twenty minutes or so? She nodded. “I’m going to ask Dr. O’Connor about adding oxygen to your heme protocol from now on. It’s worth a shot. Dr. Piersen told us a long time ago if oxygen helped you, then you should have it.” She nodded again. She was exhausted—and still in pain.
She really railed at porphyria and what it had done and was doing to her. I tried to comfort her, but she was in immense pain and scared. “My life really sucks!! I can’t do this anymore. I just can’t. I don’t want to go on,” she sobbed. Many, many porphyrics have confessed they thought that their lives were over or prayed for death to deliver them from the onslaught of the horrific pain and terrifying symptoms. Admittedly, there were times, especially before the Panhematin infusions had been figured out when I wondered whether or not Jill would make it through certain attacks.
At our next appointment, we updated APRN Kym and Dr. O’Connor about the toxin exposures she had had during the past few weeks and the very concerning recent decreasing effect of heme/D10 treatments. I showed them Ovation’s (previous Panhematin manufacturer) MSDS specifications that stated, “If breathing is difficult, give oxygen.” Then I suggested the idea of adding oxygen to Jill’s AIP treatment protocol. They agreed to try it.
All in all, Burton Academy’s use of dry-erase markers was minimal which I was convinced helped in keeping Jill’s episodes to a minimum. The combined DBT, ongoing individual counseling sessions with Vanessa and B.A.’s clinical staff had helped strengthen her sense of self. It was time for Jill to move on to her senior year. She toyed briefly with returning to Littleton to finish her education with the class she had started preschool with. But because she had ended up in the ED following the two brief times she had been in LHS while attending Burton Academy, the CIT experience and the DBT dry-marker experience, I was against her return to Littleton schools.
Summer was looming. Jill received and accepted an invitation to participate in the Hole in the Wall Gang’s teen camp. She was nervous when I dropped her off because it meant a full week away from Aaron. But Jill being Jill, she soon made friends with the campers and counselors and began to settle in. Eight and half hours later she was on her way to NHC ED with severe abdominal, back and chest pains. As I drove to the hospital, I thought that stress had triggered the problem. But when I arrived at her room, Jill reeked of smoke and told me how they had been sharing stories around the campfire when the symptoms started. I wondered aloud if the wood smoke might have had something to do with it. Jill snapped, “Oh come on mom—it was just the stress.” A few days later, I Googled and Bing-ed wood fire/AIP connection?” and found possible clues in World Health Organization (WHO) and PES articles warning porphyrics to avoid dioxin exposure. Dioxins are omnipresent environmental pollutants [that] …can present as unintended byproducts of natural events such as …forest fire.” Given Jill’s heightened sensitivity to toxins, stress and the chemicals from the campfire’s smoke could have caused her reaction.
Three years after she’d let the SFTA opportunity go, Jill ended up with Littleton-provided tutoring for the morning hours and afernoons at SFTA for her senior high school year. A creative writing class assignment was to write about an incident that ‘”all of us have had.” Jill wrote about scars. In the essay she told of her trials with illness and the vicious bullying she endured at Littleton. She explained how the tattoos on her wrists and arm had come to be. Those were the places she’ had once chosen for self-cutting. Reading her words brought pride in the young woman that Jill had become:
I went to school every day with a smile on my face but by sixth grade I stated realizing I was an outsider. I tried so hard to fit in, because let’s face it, when you’re that young, that’s the only thing you care about. If you look closer at my wrists, you’ll see the marks of my struggles from schools, bullying and my health issues. These words on my wrist tell a story. They mean something to me. It’s a promise to my friends and family—but most of all a promise to myself that I wouldn’t self-inflict wounds on my body just because I have them inside. It’s a promise to always RISE ABOVE the hurt, anger, pain, heartache, and struggles in life. And although my skin is still pale, my bones still ache and my heart is still bruised, it’s a reminder of what I went through as a victim of bullying. The tattoos remind me that I was able to get through one of the most traumatizing times in my life. It’s been over a year since I’ve cut and I feel so much better about myself. I’m not gonna lie and say the thought hasn’t come up since then, but I’ve accomplished so many goals in my life, including this one, and the person I’ve become, is a strong, beautiful, smart, young lady. I love who I am despite what anyone says.” – Jill
My daughter is on her way back–with a purple canary tattoo on her upper arm as her AIP badge of courage.
 Ovation Pharmaceuticals, Material Safety Data Sheet for Panhematin, March 10, 2008.
 World Health Organization (WHO) Fact Sheet No. 224. Dioxins and their effect on human health; http://www.who.int/mediacentre/factsheets/fs225/en/. June 2014.
 Neis, P MNS, NP. Dioxin Exposure to Be Avoided by Porphyrics. Published online at Porphyria Educational Services (PES) Monthly Newsletter, Metabolic Disorders. http://porphbook.tripod.com/Autumn2006.html. Autumn, 2006.