Priya, an HR professional, based in Thiruvanathapuram, Kerala and mother to Divya (names changed to maintain anonymity), 9 year old with a diagnosis of Juvenile Lupus shares their experience of the complexities of this condition in a young child and how she tries to let her daughter maintain a "normal" routine.
Symptoms and Diagnosis:
My daughter Divya was born in 2014. She was first diagnosed with Lupus SLE on June 15 2019 (her grandmother’s birthday) when she was 5 years old.
Divya was a very playful kid, with interests in singing and dancing, with no obvious condition till the age of 5. One fine day when I was at work, my sister noticed some drops of blood as Divya was brushing her teeth. Initially, we thought it was a bleeding of gums but then we noticed blood in her spit as well, and this we thought was definitely now a few drops. We immediately decided to take her to one of the best medical service providers around. In the meantime, we also noticed minor red rashes on her arms and the quantity of blood in the spit slowly increasing.
The hospital took her into the emergency medication area and ran some blood tests which then indicated that she had a low platelet count, and we were told she had Vitamin K deficiency or thrombocytopenia, as an initial observation. She was being then administered treatment for the same but did not seem to be getting any better.
As part of the discussions with the team of doctors monitoring her, I recollected that she did have a rash on her forehead couple of months ago but we thought that it was a rash due to heat or humidity setting in, which we treated with calamine lotion and it disappeared. Later, we came to realize that it was the Lupus butterfly rash. The only other significant illness she had until that age was a case of severe pneumonia when Divya was 1-2 years old and we were in living in the Middle East.
After a week in the ICU with no material conclusion from the diagnosis, Divya’s pediatrician recommended an ANA test.
While we were still waiting for the results of the tests, Divya once again developed a flare with high fever and vomiting (up to 20 times in an hour) at midnight. We rushed her to the same hospital and she was admitted in the ICU. Emergency tests were done, and treatment was administered to stabilize her.
3-4 days later, the doctors came out with the final findings from there diagnosis that this was a case of Lupus. My knowledge of Lupus at that point was absolutely nothing. The results of online research, which is the default means to satiate our quest for some additional information, were very scary but there seemed to be very little content that could make me feel any better, or to help me understand the condition any better and what to expect as we proceed with the treatment.
We then consulted a renowned pediatric rheumatologist based out of Kochi, who explained to us in detail about SLE and this Autoimmune disorder which can be treated and managed but without a comprehensive cure. A scan was done to ascertain if any of her organs were affected. Fortunately, it was ascertained that no organs were affected as yet. However, she explained and reiterated the necessity to start treatment without any further delay.
On the next day, as we travelled from Kochi to Thiruvananthapuram, we noticed that Divya had swollen feet. We thought it may be due to travel, with vague doubts on its possible link to the condition of Lupus as well.
The following day, Divya had fever, vomiting, severe swelling and rashes in feet again with a translucent texture on the skin where, and the veins in leg were more visible. We rushed her to the same hospital and yet again she was admitted in the ICU. Emergency tests were done and treatment was given, but there was no improvement. She hardly smiled, and we understood that she had no sensation in her feet, and neither she couldn’t stand on her own, as she collapsed trying to stand.
Brain CT Scan and MRI were done and a bleeding was detected in the brain, for which immunoglobin was administered. She slowly started getting better and was able to slowly sit upright, eat and talk, much to a mild relief in our minds. We were at the hospital for almost a month as the treatment continued.
Treatment
Initially, Divya was prescribed Steroids – Prednisolone by to manage Lupus. Later on, after about a year, her tests showed protein leakage and creatinine levels were high. A subsequent kidney biopsy revealed Stage 4 Lupus Nephritis in July 2020 and accordingly treatment was started in October 2020 for 6 months. From July 2020 till October 2020, we were at the hospital multiple times due to some infection or the other.
Steroids were discontinued after being administered for almost a year, and Cyclophosphamide, was initiated in Oct 2020 for 6 months. She is currently taking MMF (Mycophenolate Mofetil) and HCQ (Hydroxychloroquin). She is currently in remission.
She falls sick often with cough and cold due to the immunosuppressants that she is currently on and complains often of stomach pain due to gastric discomfort. She also vomits a few times occasionally.
For Divya’s medication regimen, I trust and follow our doctor from Kochi who also helps us understand the rationale for dosage and the progressive plan to treat and manage Divya’s condition. due to location constraints, we engage with the team in the local hospital at Thiruvananthapuram to manage Divya’s condition on an ongoing basis and its complexities that arise in the short term.
I do not want to try any alternate medication/treatment and risk Divya’s condition which is fortunately quite under control now.
Social, Medical and Family support
Thiruvananthapuram is my hometown where I am currently based due to family and professional commitments, and we treat Divya at the local hospital where she was brought at the time of initial diagnosis. Lupus/SLE diagnosis was finally confirmed which we immediately followed up with our visit to the pediatric rheumatologist at Kochi. We consider her as Divya’s Angel who saved her and explained the condition and helped us understand the facts to our satisfaction, to give us a heads up that we need to expect flares to happen, etc. Having said that her Pediatrician and Rheumatologist that we consult on an ongoing basis have been very supportive and empathetic to our concerns.
When Divya was admitted for a month in the local hospital, we had to face quite a lot of discouraging communication such as those on risks of MRI scans, followed by medical students approaching us for details for Case study. This we felt was a bit mentally disturbing for us or rather uncomfortable at that stage of the treatment. These were actually more demotivating than the stress of the long stay at the hospital and the complications of the treatment
From Divya’s perspective, she continued to attend school once she was comfortable and confident doing so. Due to Covid, she was attending classes online for 2 years, which turned out to be helpful in managing her condition. Last year she was a regular schooler with no major episode of a Lupus related medical emergency.
We were strongly advised to avoid physical education sessions and more specifically any exposure to direct sunlight. She takes part in all activities such as painting, dancing, etc. and remains good at academics. Divya’s school is a ten-minute drive from home and the school management and teachers have been very supportive with all our reasonable requests to ensure we manage her condition effectively. They keep us informed immediately if she is unwell, even if she has a mild fever or has vomited and have been understand if she cannot attend school if she catches a cold or has any gastric infection.
Working for me was also very stressful. My husband is based in the Middle East.
I am an HR professional with an MNC and my work turned out to be very taxing, even though I had a pretty supportive team. I was on 6 months probation period when the initial diagnosis happened and I had to manage the hospital visits and work which was very stressful. I personally have had severe gynecological issues and have also undergone 3 surgeries till date. In May 2022, I took a break from work as I was unable to handle the combined stress of hospital visits and deliverables at work.
Now, I am more composed and understand the overall situation better than before, of which the most important is being progressively educated on Lupus and how to manage as my daughter grows up.
My mother and sisters have been a huge support system have been available for any kind of support throughout.
From a financial stand point, I did blessed enough to have immense support from my employer for all of Divya’s treatment by way of Insurance cover along with a flexibility of work timings and work from home option.
Am associated with Lupus Trust India, and their Awareness Campaign and initiatives. I engaged with them recently through my sister and found their initiatives to be very useful. I want to be part of their efforts to spread the Lupus awareness. It also helps me feel that I am not alone in this endeavor to manage my child’s condition.
Emotional and Psychological impact
Till the age of 6, Divya would talk freely and mingle with everyone. She does make new friends but she is more inclined to her current friends and close family. I am noticing more shyness and an increased introvert behavior in Divya post diagnosis.
Divya is a strong girl and doesn’t like me talking about her illness. She also dislikes any kind of sympathy. Just recalling a time when I fainted while she had to go for a scan during her initial hospitalization, she was alert enough to ask me if I was ok, even in that condition.
She is not aware of the details of the medical condition, while I too want to wait to tell her when she is ready to understand it in detail. However, I have started appropriate understanding on Lupus at her age. Though she doesn’t understand why she takes so many medicines, she knew the names of all the medicines she is having and listed all of them in a school interview. She met our doctor from Kochi when she was 5-6 years old. Now she is 9 and nods her head during a recent consultation, as if she understands the situation better and the topics being discussed.
She had several bouts of anger and she even turned violent, possibly due to her condition as well as medication including steroids. She cannot tolerate loud noise which I see is a trait post the Lupus diagnosis. Relatives would never understand and suggest spiritual measures for healing. Explaining her condition to close family and friends was not much of a challenge, but it is not so when it comes to outsiders who do not understand.
We did explore consulting a psychologist as suggested by our doctor from Kochi, but wasn’t available when we tried to. I then noticed a big improvement in Divya’s behavior with the right treatment and thankfully she is feeling better now.
Divya doesn’t like to go to hospital and calls it ‘Yucky’- asks for her blood back when they prick her and take “so much blood” according to her. It still is a fear for her. However, all of the hospital staff are very supportive and are very fond of Divya and consider her a very strong and smart kid. They played a big role in saving her from her initial ordeal at the time of being diagnosed with Lupus.
When I explored if I could have a second child if I wanted, some doctors gave a very non-committal response and said it was up to me to decide. Our doctor from Kochi, on the other hand, advised me clearly on what tests I needed to take if I decide to go for it.
My message to other parents Or people in general:
Trust your doctor. Motivate your child and provide a platform to study, play and develop interest without giving in to the fear of Lupus. Don't allow Lupus to be a deterrent to let your child do what other kids are doing.
I want to talk about our experience so that people are more aware about Lupus and how it can affect children as well.
This is part of a series on Juvenile Lupus in collaboration with Lupus Trust India
Names of the patient and her mother have been changed on their request.
