ALL In: Jess vs Acute Lymphoblastic Leukemia (ALL)

October 14, 2023

Happy Birthday to the bravest, strongest, most beautiful human that my heart has ever known. This weekend we celebrated Jess' 36th birthday on the 36th day since she was diagnosed with leukemia.

After about a month of contemplation, Jess and I are ready to share our cancer journey that started at the beginning of September — and we are ready to humbly ask for some help.

On September 8th, Jess was diagnosed with Ph+ IKZF1 Mutation ALL. Simply put, Jess was diagnosed with leukemia. The type of leukemia that she has is called Acute Lymphoblastic Leukemia with a Philadelphia positive and IKZF1 gene mutation. This type of cancer is treatable, however, it is very aggressive and it has a very high relapse rate. We don’t know what caused this cancer, and it is possible that we never will. It came on rapidly and has caused a major pivot in our lives. Given the acuity and severity of this disease, I’d like to share our story to give a glimpse of what life has looked like since the beginning of August.

August brought us a lot of fun — we went white water rafting, wine tasting, spent a joyful day at Disneyland, and went to the event of the summer, The Eras Tour. But throughout the weeks of August, Jess began experiencing intermittent deep bone pain, muscle aches, radiating arm pain, significant fatigue, and some random bruising on her legs — things we contributed to the lingering effects of her most recent bout of COVID, mixed with fun weekend-warrior activities with our family and fur babies. Toward the end of August, the deep bone pain localized to her right shoulder and collarbone, but there was no specific event or injury that we could attribute these symptoms to. She has a high pain tolerance, but something was definitely wrong as she was in tears trying to put on her scrubs each morning and could barely complete a day’s worth of seeing patients. We had imaging done, all of which was non-significant. By the end of August, we were still at a loss as to what was going on with her clavicle (and body, as now the pain had spread to her right TMJ and her lethargy had increased).

We decided to make an appointment with Jess’ PCP at UCLA to do a full work-up to hopefully find some answers, but on the morning of the scheduled appointment, Jess woke up with severe chest pain that she described as an “electric ping pong ball” bouncing around her ribs. At that point, we made the decision to head straight to the ED at Hoag Hospital because we knew something was not right and needed immediate care.

The staff at Hoag were wonderful and triaged Jess right away. They ruled out cardiac involvement first, and then continued with a full work-up. Within four hours of being in the ED, we were told that Jess’ blood panel was abnormal (WBC, RBC, and platelets were all off) which triggered them to do a manual smear to examine the blood more closely. Ultimately, the PA came in and sat down with us and said she did not have great news to share. Jess would be admitted to the hospital because her tests indicated that she had a form of acute leukemia. In healthy individuals, blast cells make up 1-2% of the blood. A leukemia diagnosis is made when blast cell percentage is >20%. Jess' blood had 22% blast cells. The PA told us that the oncologist would come speak with us shortly, but that Jess would have a bone marrow biopsy later that afternoon - the bone marrow biopsy would provide information required to specifically diagnose the type of leukemia — ALL, AML, APL, etc.

Hoag kept the ball rolling efficiently and had her bone marrow biopsy done within a few more hours. We then got moved up to our room — perk of a hospital in Newport, CA was a beautiful view of Newport Harbor from our window! We took some time to ourselves, talked and tried to process the way our world had been completely flipped upside down within such a short amount of time, and then started to call family before trying to get a little sleep.

By 9 o'clock the next morning, September 8th, the oncologist we had spoken to on the first day came in to tell us that the type of leukemia Jess has is ALL (acute lymphoblastic leukemia). This type is the most common leukemia among children, however very rare in young adults. He recommended an immediate transfer to City of Hope Hospital, one of the best specialty cancer hospitals in Southern California. He shared with us that we should be prepared to be in the hospital for 4-6 weeks as Jess began the Induction Phase of her treatment. While we waited to be transferred, we started making phone calls to close friends and family, knowing that with whatever was ahead on this journey for us, we would need our people in our corner.

Our arrival to City of Hope could have been a bit smoother, but in the future, we will try to plan our emergencies so they do not happen around a shift change on a Friday night (….ha!). All jokes aside, we had a bit of a rough start at City of Hope, some of which was exacerbated by our fear and anxiety of the unknown and the lack of a medical plan given the harrowing diagnosis. What should we expect? What are the options? Should we enroll in a clinical trial? What is the prognosis? How do we reduce the risk of a relapse after only two years?! Why is this happening at all?

By the following Tuesday, we had a plan in place and had met with our incredible oncologist, Dr. Aldoss, who has the most calming, knowledgeable, and trustworthy presence. Jess’ plan for treatment was clear and it would begin that day:

- Four weeks of an IV chemotherapy drug called Vincristine

- Daily chemotherapy pills — a TKI called Ponatinib (Iclusig) for at least 34 weeks

- After the four weeks of IV Vincristine, she would then start an immunotherapy drug called Blinatumomab (Blincyto) which runs on a constant pump for 28 days. This drug requires close monitoring initially because there is a very high risk of neurotoxicity and seizures.

- After the 28 days, Jess will have two weeks “off” of the Blincyto treatment, before starting the next round.

- There will be FIVE total rounds of Blincyto completed which will last for a total of 30 weeks.

- Throughout all of this treatment, Jess will receive chemo injected directly into her spinal cord because the Ponatinib and Blincyto medications do not cross the blood brain barrier. This intrathecal procedure happens once every two weeks through a lumbar puncture procedure to prophylactically treat and prevent the leukemia from crossing into her cerebrospinal fluid.

- Bone marrow biopsies will continue to be performed every few weeks to track the progress of her disease level and ensure that she is still responding to the treatment.

- After the conclusion of these treatments, it is highly likely that Jess will require a bone marrow transplant given that her Phil+ and IKZF1 mutations are highly associated with relapse of disease. The bone marrow transplant is essentially the “curative treatment” to date for Jess’ diagnosis, and entails high levels of radiation to prepare the body for transplant and about a month long ICU stay because the risk for infection and organ rejection during that process is VERY high.

So……

When we left our house on the morning of September 7th, we had no idea we would not return home until we were discharged from City of Hope the last week of September. Thankfully, Jess was stable enough that our doctors thought it would be best for her to spend about a week and a half at home prior to returning to the hospital for another inpatient stay to start the Blincyto treatment. During those days at home, we went on wheelchair walks, caught up on snuggles with the fur babies, had visits from friends, and recharged to get ready to tackle the next round of treatment.

Remember when I said this all started because of Jess’ collarbone pain? Well, that continued throughout the entire month of September and finally our doctor agreed to a CT of the chest and shoulder. Turns out that Jess not only has leukemia, but has been experiencing pain because she has a FRACTURED COLLARBONE! The doctors are considering this a pathological fracture, indicating that the bone was so weakened by the leukemia that even a small, non forceful or traumatic movement could have been what caused the fracture. The CT also revealed some nodules on both lungs — this required a follow up bronchoscopy. We are still waiting for bronchoscopy results and praying/hoping/wishing/waiting that they are benign.

Jess’ most recent blood work showed that SHE IS RESPONDING WELL to treatment! The level of disease is much, much lower than it was prior to starting treatment, but she is still considered MRD positive. MRD (minimally residual disease) is a special test that is run at a very specific and sensitive level that can detect the leukemia cells at extremely low levels. We are hoping she continues responding to treatment and will be considered MRD negative, and eventually, in remission. From day to day, her side effects from the treatment wax and wane. Some days she is nauseous, some days she has unbearable headaches, some days she develops rashes, some days her feet and hands go numb, some days she’s so fatigued that she can barely open her eyes, and some days, it’s all of those combined, but through it all she has remained positive and her fun, lovable self.

I think that just about brings us to today, Jess’ 36th birthday. During the last month, we have felt love in the most overwhelming and beautiful way imaginable in the way people have shown up for us through phone calls, visits, FaceTimes, care packages, prayers, letters, cards, dog walks, checking in on Mama and Poppy (my 90+ yr old grandparents that live with us for whom we are primary caretakers)— truly anything and everything! We are the luckiest people in the world to have the village that we do both near and far. It brings both of us to tears when we think about the mighty love that is helping to carry us through this.

If you’ve made it this far, thank you for taking time to read our story. We’re so appreciative that you’re here.

Ways you can help:

1. Donate blood at a local blood drive! This will help other families like us who are local to your area.

2. Donate blood directly for Jess’ use when she needs a transfusion if you donate at City of Hope. Reach out to me for more info if you’re interested in this — you just need to have type O blood.

3. Sign up to be a bone marrow donor on BeTheMatch.org. This will not test to see if you’re a match directly for Jess but it will help other patients in need in the same situation we are in now. Jess will use a donor from Be The Match if/when she requires a bone marrow transplant as neither of her siblings are matches.

4. Donate here — we will have many, many medical bills coming our way. Insurance covers some, but not all of our costs. Jess’ chemo pill is $38,000 per month, and her new pump infusion (Blincyto) is $180,000 per month (…. for five months!). Her treatment will carry on for a total of 18-24 months.

5. Send cards or texts to Jess! Call or FaceTime! You are NEVER a bother. If she is awake and able to look at her phone, she’ll answer or get back to you. <3

6. Share this link with friends and family!

To know Jess is to love her - and we all have many, many, many more years left of loving our girl. Thank you for loving her this far, and thank you for loving her every minute forward.

Xoxo,

Kathryn

P.S. Jess and I will post updates as they arise to keep our tribe in the know and on the same page in this fight for Jess. Stay tuned! We love you!