LUCA'S ITP JOURNEY
Our son, the ITP Warrior, who lost his life but will never be forgotten.
Luca was an energetic, considerate, loving boy who always had a smile on his face, even when life presented him with challenges.
Luca was diagnosed with ITP, an autoimmune blood disorder, just after his 7th birthday. He had developed a rash-like outbreak on his back called petechia. The doctors who first assessed him thought he had been hurt and questioned us about potential child abuse. Bloodwork was arranged and we learned his platelet level was only 27K. Normal platelets range between 150-400K. We were told Luca would need to be hospitalized however once our hematology team was consulted, we were told that was not necessary. We were cautioned that Luca could have cancer so we were very anxious for our hematology team to figure this out. Fortunately, he did not have cancer, and no other obvious medical concerns. That first year with ITP Luca did not have any bleeding episodes and his levels averaged between 10-40K. He had mild-moderate clusters of petechia that developed off and on and large bruises that appeared with minimal injury but no real spontaneous bruising or bleeding. Not even a nosebleed. We made modifications to our daily lifestyle to keep Luca safe. We thought of ourselves as lucky. Luca was considered a non-bleeder and we followed the ‘watch and see’ approach. We were hopeful his ITP would be temporary and would resolve within a year. This was not the case. Once we knew Luca’s ITP was chronic, four-day high-dose pulse treatments of prednisone were used on two separate occasions at our request to a) establish if he would respond to steroids, and b) elevate his levels enough to travel safely. Both times, Luca responded very well. His platelet levels increased above 150K, and were sustained at elevated levels for weeks at a time. During the second year of Luca’s ITP diagnosis his average platelet count slightly decreased. He had occasional nosebleeds that were mild and lasted less than 30 minutes. He continued to develop petechia and large bruises. He developed a hematoma on his side after jumping in a pool several times wearing a life jacket.
During the third year of his diagnosis, things changed. His average platelet count was always below 10K. His platelet levels did not really fluctuate. They just stayed very low. Half-way into this third year with ITP Luca became a bleeder. During this time, Luca started experiencing frequent long-lasting nosebleeds, some of which required an emergency room (ER) visit to stop. He developed mouth blisters, gum bleeds/wet purpura, widespread petechia, lots of bruises, stomach bleed including throwing up clots, and hematuria. He developed a broken blood vessel once in his eye that we suspect was due to his low platelet levels. He started to also develop petechia on his head that would bleed and scab over. He often experienced random headaches, on average one every six weeks at least, that would quickly resolve with Tylenol. Sometimes these headaches were in the morning. He never required a CT scan since his headaches often resolved quickly on their own with the help of Tylenol and we were informed often that brain bleeds are rare.
Luca didn’t respond well to most first line treatments when he needed to be rescued from an active concerning bleed. He developed an unexpected resistance to prednisone around the time he became a bleeder. IVIG (alone) and dexamethasone (alone) did not elevate his platelet levels significantly, and when IVIG-prednisone was used in combination his platelet levels did respond well (above 100K) however his levels fell back to below 10K within a week.
We started to control his nosebleeds and his mouth bleeds with tranexamic acid. We were told in January (2018) that Rituxan would be an option for Luca, however our government in Ontario (Canada) was unable to approve funding for this drug and we were unable to access it before Luca passed away through alternative avenues. Luca did not receive any treatment after the first week of February (2018) despite his low platelet count because Rituxan did not come through. Apart from trying dexamethasone in early February, Luca was only treated when he was having an active serious bleed. Towards the end of March (2018) Luca had a routine eye exam and was found to have intraocular pressure bilaterally. We then became worried about glaucoma. We wondered if it was due to steroid use although he’d only had short pulse treatments at high doses. From February May (2018) he did not have any major serious bleeding but he continued to develop during this time widespread dense petechia, lots of sporadic ugly bruises, frequent nose bleeds that were not long in duration but sometimes happened up to four times in one day. By April, Luca had a few tiny blood blisters on his lips most of the time.
In May (2018) Luca developed a fatal brain bleed. Luca’s brain bleed symptoms (we believe) started a day before he had his confirmatory CT scan. Unfortunately, Luca’s symptoms were interpreted as a sign he was sick with a bug likely because he didn’t present in a classic expected way. Luca presented with a mild headache that resolved with Tylenol but kept coming back. He had a lot of fatigue and no appetite. His headache hurt more when he moved his head in a certain position. His headache was light sensitive, but not progressive. We were always told to look out for a thunderclap headache that got worse that didn’t respond to Tylenol, and was described as the ‘worst headache ever’. That is not the type of headache Luca experienced. Over-night Luca threw up twice. He went in and out of sleep the following day. He didn’t complain of a headache in the afternoon the day his brain bleed was detected. He didn’t want to eat or drink. He only took miniature sips of liquid that morning. He started slurring his words by mid-afternoon. By the time Luca got to the ER approximately fifteen minutes later he was showing weakness on one side, and then soon collapsed at triage. A CT scan revealed a significant brain bleed with increased intercranial pressure.
We are not sure what his platelet count was at the time of his brain bleed, but five days earlier, his platelet level was less than 5K. It took over five hours before he could have surgery because his platelet level was too low. We were told platelet infusions were not able to increase his count, so at some point dexamethasone and IVIG as a combo was given to him. Around 10:30pm that night he went for surgery, a left-sided craniectomy was performed to reduce the pressure. Before surgery his left pupil was fixed and we were told that was a bad sign. They were only able to get his platelet levels up to 96K prior to the surgery. Around 1:30am the following day he returned from surgery. The surgeon was hopeful and said his left pupil became unfixed and they were able to control his pressure. Luca has always been a warrior, so we believed he was fighting hard to stay alive. Unfortunately, his pressure spiked again hours later, and he required a second craniectomy. When he came back from surgery this time, he remained unresponsive. We were told Luca would not recover. A few days later, his nuclear scan combined with the physical brain death assessment confirmed Luca had no blood flow to his brain. We took him off life support on May 15, 2018. He was only 10 years old when he died.
We are unsure if his brain bleed was spontaneous, or due to the pressure from a bike fall he had less than a week before his brain bleed (he was wearing a helmet), or other casual effects of active play.
We are proud to host our 3nd Annual Pump it Up for Platelets 5K walk/run in London Ontario this year in honor of our ITP warrior, Luca. All funds go directly to support the Platelet Disorder Support Association (PDSA), a place that has supported us tremendously through the years. Money raised will be used to further education, advocacy and research in the area of ITP. The event will take place virtually once again this year due to concerns about COVID-19. You can walk your way while raising awareness for ITP.
PDSA is a 501 (c)3 organization.
Contributions are tax deductible to the extent of the law.
PDSA is eligible for corporate matching programs.
PDSA receives NO federal funding.