Spotlight on AHC Champion Dee Dee
Here is the spotlight on AHC Champion Dee Dee (AR, USA), as told by her mom Mandy. Dee Dee recently attended the AHC Clinic at Duke Children’s Hospital.
When I found out I was pregnant with Dee Dee, my husband and I thought it was a miracle. Our oldest was 7 and we had long ago accepted the fact that she would be an only child. After Dee Dee was born, we could tell that something was different from our oldest. She cried constantly, if she was awake she was crying. We thought it might be colic, so we tried everything, and nothing helped. When she was 5 weeks old, I got up in the middle of the night to feed her, and since she was still asleep I was taking my time and being slow about it, because she didn’t usually stay asleep for very long. I started watching her there in her bassinet, and noticed her chest wasn’t moving. I looked closer because I assumed that I was too sleepy to notice, then I realized she really wasn’t breathing. I screamed for my husband, and we began to give her rescue breaths. The rest of the night was a blur, but our local hospital sent us to the Children’s hospital, and that is how our journey began.
Dee Dee had Lyrangomalacia, and reflux, so everything that happened was attributed to those two things. She would go limp, pass out and her o2 sat would drop. She would stop breathing daily. As a toddler it was evident she had some developmental delays and sensory processing issues. No matter how much research I did, she never quite fit into any particular diagnostic “box”. There would be days that she could no longer do something she had been able to do previously. There were days her face would droop, and she would look like she had had a stroke, or her eyes wouldn’t quite line up. She would often turn blue, or become really hyper and run around, only to pass out the moment she sat down. She had retained her primary reflexes,
She had a huge list of “allergies” Strange ones, that caused her to have breathing issues or to pass out, not like a normal allergy would present. I remember telling her doctor that it was people that have seizure triggers, her brain just goes haywire when she is exposed to certain things under certain circumstances. Like if there is cigarette residue on someone’s clothes, furniture, or even in a room, she would get very sick and it would take two weeks for her to recover. She didn’t need to come into contact with the surface, just being in the same vicinity was enough. Once she got sick because we had some clothes that we had bought from a sidewalk sale, that had been placed where people were smoking outside the entrance. But we could walk past those people as we entered the store, and she would be fine.
Not quite “allergies” but we weren’t sure what they were….
The next few years we started trying to find out what was going on with our little girl. We went from specialist to specialist who all said the same thing, it’s not structural, it is neurological. But she didn’t have seizures, so the neurologist said she was fine. Other doctors would try to help her, and run test after test, but send us back to the neurologist, saying there was something going on in her brain. She was in and out of the hospital, each time they would run tests and come tell me what they ruled out and send us home. “Well It’s not Seizures, you can go home” I asked what it was and there was never an answer. Her therapy team tried to let them know what was going on her, she would have weakness on one side of her body at times, but a different side the next time. I wanted the doctors to see I wasn’t fabricating her symptoms, I wasn’t the only one who saw them. Because I often got the question “Has anyone else seen her do this?” Finally I had the hospital’s Physical therapist come work with her. He did several things and said she was fine. I told him to check every part, make sure he had seen everything, because tomorrow something would be different. Something would be weak. He humored me, and the next day she was unable to hold herself up on a ball. He had never seen anything like it in his life. I was so thankful because now their own staff could attest to her having transient spells of weakness. The doctor said he believed she just wasn’t trying very hard.
I felt like he spit in my daughter’s face.
Her regular neurologist said he felt it was something she would grow out of soon, that she was just taking a little longer to develop, and a diagnosis was not really necessary since she was already doing OT, PT, and Speech, and that is all that they could do for her anyway.
On the day she was discharged, I pressed the attending doctor for an answer, and all I got was that it wasn’t seizures. I told her I knew that before we were admitted for all these tests, Seizures weren’t on the table, we were here to find out what she actually did have. She just looked at me and said they couldn’t test for anything else, the only other test they could do was a neuropsych evaluation.
It was then that I knew the children’s hospital could not help us.
So I brought her home, and took her to our family doctor. I told him we needed to find another option for her. I had two ideas of places I thought could help her, but we were all exhausted physically, emotionally, and financially, so we were going to take some time to make sure we were taking the right path this time. I remember him telling that sometimes parents think that the next doctor or test will have the answer, and they may, but they may not. Having been our doctor since I was pregnant with my oldest, he has had to have a lot of tough conversations with me. I knew I might have to accept and learn to live with not having an answer. I told him, that may be true, but when she was grown, I would have to look her in the eye and tell her she got my best effort, not the first cousin of my best effort.
I don’t know how he did it, but in four years of trying to get her in a with a diagnostician, he had her in that office two weeks later.
I want to stop and make sure to acknowledge that during that four years we had doctors who went above and beyond to try to help our daughter. They did more than what was expected of them in the areas of kindness and caring. I want to make sure that even in my worst moment of frustration of a lack of answers, as a person, my daughter was always treated with kindness and respect. Her ENT and Sleep Clinic Doctors spent time outside of her visits, to try to figure out her puzzle. Our Family doctor would sit with me and patiently hear my list of “could it be….” And talk me through why it wasn’t. Her therapy team would come back from conferences with new ideas of things that might help my daughter. During this time, I was not the only one searching for an answer. Our family wasn’t dismissed or uncared for. We didn’t have incompetent people surrounding us. She just did not fit into any diagnostic “box”
At the diagnostic clinic, the doctor was already well versed in Dee Dee’s symptoms and history, so she really only had a few things to ask. She looked Dee Dee over, and said she thought she knew what it was, but wanted to confer with two other doctors. She came in and told us she thought our daughter had Alternating Hemiplegia of Childhood. So I googled it on my phone, and as I read the description I recognized the struggles my daughter had gone through. It was like everything fell into place.
The first thing I felt was relief. I knew the name of the dragon I was fighting.
After that, there were three things that kept coming up as I educated myself about AHC. Debilitating, Progressive, and No Cure. Those stuck in my head. Another thing that kept coming up was a clinic at Duke University. So I just called one day to see if I could get my daughter in.
In August we were up for an appointment at a Developmental Center that we had been on the waiting list for 18 months. It took that long because we needed an all-day comprehensive evaluation of our daughter by several professionals, along with developmental testing. We were hoping for answers to where my daughter was as far as development. Her Therapy team hoped they might have a new strategy or ideas to help Dee Dee. My husband took off work and we took the 5 hour trip to the center. Four people came in and met with us, we told them about her new diagnosis with AHC and some of the things she experienced. We waited for about 45 minutes as they decided what evaluations to do with her. When the doctor came back, she too sent us home. There was no need. She has AHC. She wasn’t autistic, and did not have behavior problems, she was not defiant. But she had AHC so there was nothing to do for her. They suggested she see someone about adaptive equipment, and have a neuropsych evaluation in a couple of years.
I wasn’t sure if they knew something I didn’t, maybe there was nothing to hope for, for my daughter and we just needed to make her as comfortable as possible and not expect much. But my daughter, like I have found other people with AHC to be isn’t one to be limited. She finds ways to adapt and get things done.
I reached out to Cure AHC and was thankful for the correspondence and realistic look at what my daughter’s future holds. We replaced our steps with a ramp. And made changes around our home so that as her abilities vary from day to day, she can still have some stability. As a mother it was also great to have a place for my “Is this supposed to happen?” questions to go while we waited for our appointment at Duke.
During the middle of that we would have times of panic. I was on away from home for a training with my job when I got a call that out of the blue, my daughter had gone blind in one eye for a time, but her vision returned. I reached out to those that I knew from Cure AHC. I have to say, I had more than one panic moment that I had to be talked through.
When our first appointment arrived, I went in armed with binders of medical records because I was used to having to show someone else’s documentation of my daughter’s experiences, because some of what happens in an episode just sounds crazy to most people. I expected the appointment to go something like this. We come in, the doctor looks over our daughter and we spend the rest of the appoint with me trying to convince him that my daughter really needed to be seen. I expected we would get a polite smile and be sent back home without much difference in her care plan.
That was not our experience. I stuttered through most of our appointment because I was not prepared for the questions Dr. Mikati asked. I was ready to defend our reason for being there. To explain that I know sometimes it’s her right leg, but other times it’s her left. Really, I’m not crazy. Like a UFO sighting, I had letters from people who had seen it too. One day she could kick a ball with the side of her foot, but another day she couldn’t make her leg move that direction.
There was nothing to explain. Dr. Mikati was familiar with her chart before he walked through the door. He wanted to know how often was it her left foot as opposed to her right. I didn’t know. I had never gotten past the fact that the problem actually existed with a neurologist. Systematically he went through her symptoms from head to toe. All the craziness didn’t surprise him at all. In fact, he knew what it was and could explain it to me why it happened.
I was not at all ready for answers.
Then he asked what questions were on my list of things to talk to him about. He had covered most of them already. Then he said, he thought they could do something about her central apnea, saying she doesn’t have to live with that.
I hadn’t even brought her apnea up. I didn’t think there was anything that could be done. I didn’t even hope there was something that could be done.
He said the blindness isn’t an episode. It’s a hemiplegic migraine. The medicine he was giving us would fix that too. I wasn’t ready for that either.
After the appointment, I wasn’t sure what to do with all the energy still in me that had been earmarked for fighting with the doctor, but I went home with hope.
We started Dee Dee on the Flunarizine, and over the next few months we had some ups and downs. Days I thought she was getting better and would be fine, then she would have an episode and I would wonder if she wasn’t getting worse and would ever live independently. Our mindset had to be shifted from thinking she would outgrow this, to this was our life now.
Soon it was time for us to be seen at the multidisciplinary clinic. It was a week-long marathon of appointments ending with a three day stay in the hospital for a video EEG. We were met at the hospital by the AHC coordinator there, who gave us a folder of information and helped us find where we were going.
The benefit of having a clinic like this is that the medical staff isn’t surprised by the things that happen, or the history you give them. There was something comforting in hearing “yes, I’ve seen that” And the even more outstanding “Here is what you can do to help her when this happens” After the first day, I didn’t even bother to bring the binders of her past medical records. We got encouragement, support, measurements, answers. My daughter’s skin sometimes becomes mottled and turns blue, it turns out that’s autonomic and not “well, she’s just fair skinned” She has twitches and tremors, that is part of the dystonia, not “it’s not a seizure, so it must be nothing” My concerns were replaced with answers. Even the scary things were made easier just by the lack of surprise on the doctors’ faces. I was also impressed that during the 3 day video EEG when I would push the button, the team would already be familiar with the event when the doctors came around the next morning. I would say, her heart raced yesterday and a doctor would say something like “do you mean the one at 11:33?” without even looking at his paper.
The other thing I was impressed with was the extent that they went to to make sure my daughter was not in pain, even for blood draws. I mentioned her IV had become painful, and the nurse said no problem, and put it in her other arm.. Problem solved no fuss. I was very worried about her having a spinal tap, but that didn’t last long when the anesthesiologist took time to talk to me about the procedure, and not just the risks involved, but what would be done if something did happen. If she has trouble breathing, I will do this, if this happens, we will do this. She may have vivid dreams, so before she goes to sleep we will ask her what she wants to dream about, and most likely that is what she will dream about. I have never been in a hospital wither they were that concerned about my daughter’s well-being to try to circumvent nightmares. There was an allergy poster on her door, when people would enter, they would verbalize that they had checked at the nurse’s station before entering. One of the doctors took time to talk to me about my own well-being, as did a social worker not just for my time there, but long term. I truly have to say that it was worth the 1600 mile drive. It’s also a whole new world for our family to get all of her medical visits done in a week, instead of making a 5 hour drive one way every few weeks. It’s life changing that Duke can just coordinate with our family doctor if needed so we aren’t traveling all the time.
We left with a plan, a team, and hope.
Today we still have good days, and hard days, but she has a full life with hopes and dreams and goals. On the days she can’t walk, she rolls. If she can’t chew, we make a shake, If she can’t make her bed, she trades chores with her sister for the day. If she can’t hold her fork, we do finger food. If she can’t write that day, we do her lessons orally. If it is a really bad episode, we just take the day off. We still do what we do, we just do it with AHC.