Q & A with Lorelei

This week, instead of a topic from my perspective, I thought it might be interesting to read about someone else’s experience. Lorelei has anterior uveitis, associated with Juvenile Idiopathic Arthritis (JIA). I’ll link some sites about JIA at the end.

I met Lorelei’s dad through work before she was born. I remember how scary it was when Lorelei was diagnosed and I couldn’t believe that she also had such a “rare” disease. 10 years later we even see the same doctor. Meeting her, you would never guess she battles an autoimmune disease; it definitely isn’t stopping her!

Lorelei Burnett, 13

About you:

Hobbies: Playing Soccer

Last book you read: Unpregnant

3 things you can’t live without during #stayathome: As much as I hate to say it my phone, a soccer ball and my dogs

About your uveitis:

What type of uveitis do you have / what is your diagnosis? Is your uveitis associated with another condition or disease?

I have Juvenile Idiopathic Arthritis (JIA) which caused my uveitis.

How did you know something was wrong? When were you diagnosed and how long did it take? 

When I was first diagnosed I was three years old and my parents had noticed that my eye was red and inflamed. They took me to the doctor for what they thought was pink eye, but  when the antibiotics for pink eye didn’t work and the different allergy medications they tried didn’t work, they finally decided on sending me to a child ophthalmologist who was the one who diagnosed it.

How are you currently treating your uveitis? What other treatments and/or procedures have you tried?

 When I was first diagnosed I had two surgeries one to remove the lens in my left eye which had a cataract on it. ( At that time I was also one a lot of different steroids which with uveitis can sometimes cause cataracts.) And then another surgery to remove scar tissue from the first surgery. Currently I take Humira which is an immunosuppressant which keeps me from having flare-ups. It’s a bi-weekly injection that I can give myself at home. I also take methotrexate which is a chemotherapy drug which stops me from having reactions to some of the other medications. In the past I was on remicade which you have to take intravenously so it was really hard to go to the hospital and miss so much school.

Do you have flares? What are they like?

When I’ve had flares in the past for me I’ve never really been able to tell when they were happening, my JIA, except for when I was really young, has only affected my eyes so for me there is really no tell-tale sign that I’m having a flare-up. They’ve happened in the past when they’ve been trying to wean me off my medication.

What is the hardest part of having uveitis for you? 

Honestly I’ve been really lucky it really doesn’t affect my daily life. I guess one of the hardest things in the past was having to go to the hospital sometimes having three weeks to get my IV’s. It was hard because I missed a lot of school between IVs and opthamologist and rheumatologist appointments. But since I’ve switched to at home injections it’s been so much nicer. One thing that’s always been super hard is taking the methotrexate when I was younger it was always a battle between my parents and I to get me to take it. It’s really hard on your body and I would end up throwing up most of the times I took it even though I was at such a low dose.

How has having uveitis changed your life?

Aside from doctors appointments and medication it really hasn’t. I can still play sports and do everything someone without uveitis would.

Do you research uveitis (causes, treatments, etc) or get all your info from your doctor?

I’ve researched a lot about it in the past, I actually did a whole presentation on uveitis and JIA for one of my classes this year. 

Do you think people know about uveitis? What do you wish people knew about uveitis?

 Honestly I don’t think a lot of people know about it. I’ve tried to explain it to my friends and when they hear about my surgeries they either get grossed out or I get met with blank stares. I really want people to know that kids get uveitis and arthritis too, in fact JIA is actually more common than childhood type 1 diabetes.

Anything else to add?

I guess I just hope that anybody going through a diagnosis or living with it right now knows that they’re definitely not alone. I also hope everybody is staying safe and staying healthy right now at this crazy time.

Read more about Juvenile Idiopathic Arthritis and associated Uveitis:

Arthritis Foundation: Juvenile Idiopathic Arthritis (JIA)

OIUF: Juvenile Idiopathic Arthritis and Uveitis: What is it and what is its effect on the eye?

Uveitis associated with juvenile idiopathic arthritis

Women and uveitis / my pregnancy journey

Women make up the majority of uveitis patients, young and old (Faia and Drenser, 2017). In developing nations this is likely due to lack of access to medical care when the uveitis progresses as a symptom of an infectious disease. In the United States, however, non-infectious uveitis is more prevalent (80-90% of cases) (Yeh and Shantha, 2016) and is most often associated with autoimmunity which occurs far more often in women (Fairweather and Rose, 2007).

The first time I saw flashes was when I ran a Jingle Bell run in December 2009. It was cold and thought maybe I was pushing my body too hard. Then in Costco a few weeks later, the lights were reflecting oddly to me, like there was a glare or a smear over my lens. Only, I had undertaken LASIK surgery a few years earlier and had perfect vision. I stopped in the optometrist’s office before it closed and luckily he had time to see me. He looked into my eyes for a long time. “Is your dad with you?” was the first thing he said to me and I knew something was really wrong. I was 27, living with my fiancé, an hour away from my dad who would have supported my reply which was “No. And they’re my eyes, please tell me what’s wrong.” He said he wasn’t sure but that I need to see my ophthalmologist the next day; it was an emergency. 

I cried all the way home. Test after test came back negative (which was a relief but also frustrating) and my condition as declared “idiopathic” (unknown cause) which seems to translate into autoimmune or autoinflammatory in the minds of many, even if the initial cause is unknown. 

The reason for the higher occurrence of autoimmune disease in women is complicated and not definitively understood. It is thought to perhaps be due to issues with deactivation of genes on the X chromosomes. Since women have two X chromosomes and don’t need two copies of each gene, one of each gene is supposed to be deactivated; when this doesn’t happen in an evenly distributed method, instances of auto-immune disease are more common (Ørstavik, 2017). 

Another line of research suggests that female hormones contribute to auto-immunity. Proper functioning of the immune system requires a balance of Helper Cell Th1 and Th2 (see Immune Page). After puberty and until menopause (two milestones that vastly change hormones, which in themselves can throw the body out of balance), a woman’s world in cyclical in nature, with hormones in flux in phases. Estrogen and progesterone promote the anti-inflammatory response and therefore suppress pro-inflammatory cytokines (signal proteins). A study conducted by C. Sanghvi, K. Aziz, & N. Jones in 2004 “demonstrated a significant increase in the incidence of Acute Anterior Uveitis arising late in the menstrual period.  We postulate that the withdrawal of either or both [o]estrogen and progesterone provokes the onset of inflammation.”

The anti-inflammatory response happens during pregnancy as well. A rise in estrogen and progesterone signal the body to “tolerate” the foreign invader (growing fetus!) by suppressing the Th1 response. This can worsen symptoms in auto-immune diseases associated with Th2 excess (such as lupus) and relieve auto-immune diseases associated with excess Th1 response (psoriasis, MS, rheumatoid arthritis) (Young et al., 2015) Some research includes non-infectious uveitis in the latter category (Yeh and Shantha, 2016).

My fiancé and I got married nearly a year after my vision problems started with no answers in sight (ha…) but nothing seemed to be progressing. By this time I had seen a handful of doctors and before being referred to the University of Washington Eye Institute, which is where I am still seen. To get me off of the high dose of prednisone I was taking (which is pretty miserable, not to mention unsafe long term), my doctor prescribed steroid-sparing drugs (cyclosporine, methotrexate) which had a whole host of different side effects. A major side effect for me was that I was unable to get pregnant on them. 

In 2012, I had surgery to receive a RETISERT implant (a time-release steroid implant in the eye) and a new lens, and weaned off of systemic medication. A year passed and I wasn’t pregnant. Meanwhile my left eye became active and my right eye pressure slowly climbed despite medicated drops. In 2013, I had another surgery to insert an Ahmed valve to reduce intraocular pressure due to the steroids in the RETISERT. 

In 2014, I started seeing a naturopath who put me on a low dose of thyroid medication, increased my progesterone, and within a month I was pregnant. “Glucocorticoids such as prednisone can lower serum Thyroid Stimulating Hormone (TSH) levels and decrease TSH secretion… however do not induce clinically evident central hypothyroidism even after prolonged high dose use,” according to Haugen (“Drugs That Suppress TSH or Cause Central Hypothyroidism,” 2009). Does prednisone cause enough of a suppression that patients may need micrograms of levothyroxine (thyroid hormone) to reach optimal levels or was this a separate issue with my body? Yet another unsolved hormone mystery. 

My obgyn was a doctor in my small city who had never heard of uveitis and I felt very judged about the 10mg of prednisone I was on throughout my pregnancy despite research I had read pointing to an extremely low chance of side effects (as is true with many things you expose your  body to while pregnant). The literature is even more “forgiving” today than when I was pregnant (Read: Prednisone | Prednisolone – MothertoBaby.com (2018) and A review of systemic corticosteroid use in pregnancy and the risk of select pregnancy and birth outcomes (2017)). Of course no one wants to be on medication that makes pregnancy even scarier than it already is, but I weighed the risks of my dose.

The intraocular pressure in my right eye had gone up again during my pregnancy; my body had developed scar tissue virtually blocking off the entire valve. My vision was pretty bad in my right eye at the time and I can remember my face growing hot with humiliation when I would go to pick up my son at daycare and not be able to pick him out of the group! Finally I let the teacher in on my issue (we are still friends to this day although neither of us are at that daycare any longer). From then on she would proclaim “here he is!” with a smile when I arrived and I wondered why I was so hesitant and prideful to not tell her initially. When my son was 5 months old, I had a trabeculectomy.

The night after the surgery my mom stayed with us to help. She and my husband took turns waking with my son as I pumped and dumped my breast milk due to the pain medication I was taking. (Yes, I breastfed on a low dose of prednisone. LactMed is a great resource for medication use during pregnancy; there are applications for mobile devices as well). I remember thinking that night, which was one of my longest nights, with my breast pumps going, my son crying and my eye pulsing in pain, that I just had to get through to morning; I knew that night was temporary even if my disease wasn’t.

I got pregnant faster than I imagined the second time and 22 months after the birth of my first son, we had another little boy. Dealing with the medication, check-ups, procedures, and often urgent nature of uveitis while having small children is definitely challenging, but it can be done with support.

My uveitis flare-ups, unfortunately, have yet to cease and maybe never will. While picking up my steroid prescription with me the other day, my 5 year old asked what the medicine was for. I told him,” for my eye.” He asked why. I told him, “sometimes my eye just needs a little help”; a simple answer for the most complicated part of my life. “Oh, ok,” he responded and we held hands back to the car.  -January 20, 2020

Works Cited

Faia, Lisa J. and Kimberly A. Drenser. “Pediatric Uveitis: Challenging for Ophthalmologist, Patients and Parents,” Retina Today, October 2017, retinatoday.com/pdfs/1017RT_Cover_Drenser.pdf.

Fairweather, DeLisa, and Noel R. Rose. “Women and Autoimmune Diseases1.” Emerging Infectious Diseases, vol. 10, no. 11, 2004, pp. 2005–11. Crossref, wwwnc.cdc.gov/eid/article/10/11/04-0367_article.

Haugen, Bryan R. “Drugs That Suppress TSH or Cause Central Hypothyroidism.” Best Practice & Research Clinical Endocrinology & Metabolism, vol. 23, no. 6, 2009, pp. 793–800. Crossref, www.ncbi.nlm.nih.gov/pmc/articles/PMC2784889.

Ørstavik, Karen Helene. “Why Are Autoimmune Diseases More Prevalent in Women?” Tidsskrift for Den Norske Legeforening, 2017, pp. 1–6. Crossref, tidsskriftet.no/en/2017/06/kronikk/why-are-autoimmune-diseases-more-prevalent-women.

Sanghvi, C., Aziz, K. & Jones, N. Uveitis and the menstrual cycle. Eye 18, 451–454 (2004) doi:10.1038/sj.eye.6700713

Yeh, Steven, MD, and Jessica G Shantha MD. “Retina Today – The Burden of Noninfectious Uveitis of the Posterior Segment: A Review.” Retina Today, Bryn Llc Mawr Communications, July 2016, retinatoday.com/2016/08/the-burden-of-noninfectious-uveitis-of-the-posterior-segment-a-review.

Ian YL Yeung, M.D., Nicholas A Popp, B.S., and Chi-Chao Chan, M.D. The Role of Gender in Uveitis and Ocular Inflammation. Int Ophthalmol Clin., 55(3): 111–131, 2015 Summer doi: 10.1097/IIO.0000000000000072