Rare Disease Day

When Jaxon was diagnosed with Acute Lymphoblastic Leukemia almost two years ago ... it was the darkest day of our lives. Paul and I mourned the news, grieving that there was no easy answer to our questions of "why?" and "how?" and "what if?" and "what now?". Not long after the news of his diagnosis, I was struck by a thought of gratitude. We had learned WHAT was making him so very sick and we knew that there was a thorough and highly effective strategy for treatment. The reason the diagnosis of Jaxon's type of Leukemia has gone from 100% mortality rate to 85% expected long-term survival is because of research. Childhood cancer (collectively) is not rare, though many sub-types are extremely rare. Although A.L.L. has become the most optimistic of childhood cancer diagnoses, (some call it "the good one" - no. such. thing!), cancer in general is the reason for the most disease-related deaths of Canadian children. Lots of kids get A.L.L. so there are lots of opportunities to study it and to improve treatment for it. But there is still so much to learn and improve for the treatment of all childhood cancers. Due to low numbers of kids getting rare types of cancer - funding for research is minimal.

On Jaxon's diagnosis day I thought of my friend who had been taking her young daughter to multiple specialists for several years, trying to get an explanation for her frightening and mysterious symptoms. Their family was living under the shadow of uncertainty for an agonizing length of time. Although we were facing a lengthy recovery time for Jaxon with the potential for complications at any time, I remember feeling grateful that AT LEAST we knew what this was and somebody knew what to do to help. Not every parent in similar situations has this in their favour.

Some would say that cancer is every parent's worst nightmare. I don't fully agree. Yes, cancer is heinous. There is absolutely no denying or minimizing or sugar-coating it. Diagnosis and treatment of Leukemia has been OUR family's most difficult struggle. For many of my friends - more rare and aggressive and recurring pediatric cancers have brought devastating physical and emotional effects and death - painful beyond comprehension! For others, a rare disease causes unrelenting symptoms for which there is little to no relief. The prospect of a shortened life expectancy, endless watchfulness or progressive debilitation is excruciating for the families who love a child with a rare disease.

Whether it's a common/well-known disease or a rare one, a chronic illness or an acute crisis - I believe the worst prospect any parent faces is that of losing their child, or watching their child suffer or having to cope with significant difficulties and not being able to do anything to relieve the burden. It could be Leukemia or Ewing Sarcoma or a hundred other sub-types of pediatric cancer or a traumatic brain injury or Wiskott-Aldrich Syndrome or Spinal Muscular Atrophy or Eosinophilic Esophagitis or severe allergies/asthma/eczema or Epilepsy or Congenital Heart Defect or Opitz Syndrome or Denys-Drash Syndrome or Hypophosphatasia ... or ... or ... or ...

The severity of one experience does not diminish the impact of another illness or injury or difficulty faced by another family.

My friend said to me the other day "There's no such thing as 'other people's kids.'" It could happen to anyone. We all need the support of others. I am honoured to journey with families like these in their most difficult trials, just as they and others have done for my family. I raise my voice to support them through awareness to bring about more meaningful research toward better treatments and cures. I will take them a meal or a cup of coffee. I will give blood. I will say "yes" whenever possible when asked to donate money to support a family in need or a local children's health initiative. I will ask others to do the same.

this is the face ...

... of a very unhappy boy!

Jax started coughing a really harsh dry cough on Monday morning. We already had an appointment at our clinic in Victoria for later that afternoon so I wasn't too concerned. He just had a dry cough and no other questionable symptoms. He handled his ventolin and inhaled antibiotic really well and we made our way to Jeneece Place for the night. He had a good night and we went to his chemo appointment as planned on Tuesday morning. It was a long day with an LP and IV chemo. Nurses and Doc listened carefully to his chest and it was all clear, so they advised me to just watch him carefully as the week went on, especially after his Dex wore off early next week. (Dex elevates his white blood count, so once the elevation wears off, viral illnesses can become more intense.) The cough lingered and then on Thursday he started having a stuffy nose. Since he's always more sensitive and moody while on Dex, adding in the illness has made him a very grumpy bear.

Friday night he hardly slept at all. Coughing non-stop. Ever since he was just little, he tends to cough so hard that he vomits. That's been in full force this week too. Yuck. He was so miserable last night, and we couldn't give him anything to help him feel better, since Tylenol is a no-go for someone with a VAD. We can't give it because it can mask a fever which could lead to missing out on catching a bacterial infection. A bacterial infection can be very dangerous for someone who is immune-suppressed. 

Because his VAD line goes directly into his heart, 

we have to take fevers very seriously. Every time. 

He begged for the morning to come so he could get out of bed and lay on the couch. He finally napped late this morning but when he woke up I could tell his temperature was significantly elevated. I had checked a few times through the morning and he was always within normal range and this time when I checked it was 39.4. I've never seen his temp go that high so I jumped into flight mode. I grabbed a few things, put Emla (numbing cream) on his port and got him packed up to go. Paul had taken the car into Lake Cowichan so I called him to get home right away. He swooped in and helped me get everything ready to go and called the hospital to let ER know we were on our way. 

Once we got there he was seen by triage right away and his temp was a bit lower but hovering very close to the threshold to begin what's called Clinical Order Set - Fever and Neutropenia Protocol. Long name with a bunch of steps to take for a chemo patient with a fever. We were soon given a private room and nurse and doc saw him right away. His temp was still so very close to the magic number that they decided to begin antibiotics. He got some tylenol and codeine (supposedly has cough suppressant properties), chest x-ray (looks good), and first dose of prophylactic antibiotics to cover any bacterial infection that may be brewing. He was seen by the pediatrician on call, who agreed with the ER doc that it's a standard cold virus but protocol would continue until blood cultures have been 48 hours to show any signs of infection.

The nurses decided not to use his VAD port for drawing the bloodwork. I was happy they didn't want to access his port unless necessary, but not happy about having to tell Jax that he'd be getting an arm poke without any numbing cream. He agreed to it on the promise I gave that the lady knows what she's doing and it wouldn't be as bad as he anticipated.

It was bad. 

She missed his vein the first time and spent a minute re-aligning to try to make it work, but to no avail.  The plan after that was to give Jax a break, put Emla on the other arm, and have a different tech come attempt the IV. 15 minutes later that happened and she got it on the first try.

Not long after that we had to get going with accessing his VAD. Sadly, the Emla I had put on it at home 3 hours prior had lost its potency so he felt the pinch on that one also. 3 pokes with very little numbing is hard for a boy who usually doesn't have to feel much from pokes. 

As grumpy as he was, he was rocking some totally rad bed head.

Once the meds kicked in he started to feel much better.

We're thankful this didn't happen in the middle of the night, thankful that this is a rare occurence for us (only his third fever since diagnosis in April 2014), and thankful it seems to be viral and he'll come through just fine. Many oncology patients and their families spend countless days and nights in the ER and admitted to hospital, dealing with serious infections and complications from the powerful medications used to attempt to restore these kids to health. I just found out that our little buddy Marek was in ICU for 9 days this past month. That could just as easily have been our situation.

As I write this he's been asleep for about 2 hours with just a few coughs. In a strange twist ... now that I'm actually allowed to give him Tylenol to make him more comfortable (because he's already getting antibiotics and blood cultures have been drawn) ... he's refusing to take it. He did accept a Gravol though, so there's that. I can tell he's having a good sleep because he's doing the cute little sigh-coo thing that he does when he's sedated for his LPs.

After his LP on Tuesday, in his loopy state, he said "That was fun! That was the best back poke ever! Mom - you have FOUR eyes, TWO noses TWO moufs ... and ONE CHIN." (Yay, just one chin!)