MDS Triumph: Fran Boyle’s Journey of Hope and Healing

MY STORY – FRAN BOYLE

My MDS Journey started in 2000 when my family doctor noticed my haemoglobin decreased slightly noted in a CBC test reported during my yearly physical. After this occurred three years in a row, he referred me to a local haematologist in 2003. He told me he didn’t know what to make of it, so I would return to him yearly until he decided to do a bone marrow biopsy. That was in 2006. I was 56.

Results showed that I had RARS. Refractory anaemia with ringed sideroblasts. My CBC showed that I had a haemoglobin level of around 10-11 at that time.

The haematologist didn’t give me any information; he just told me not to look it up on the internet as I could have this for years without needing any treatment. It was only after surfing the web that I found out that what I had was considered a blood cancer. Some haematologists don’t know much about the disease! While searching the web, I eventually found the MDS Foundation and realised I needed to attend an MDS Center of Excellence.

The consult

I consulted with Dr. Porter at the University of Pennsylvania, where I had my second bone marrow biopsy, which had the same result – RARS. They suggested testing two of my four brothers to see if either of them might be a match for a bone marrow transplant. Up until then, I was reluctant to tell my daughter, family members, and friends about my diagnosis. I didn’t have any idea what direction my MDS would take.

I then saw a specialist at Thomas Jefferson, Dr. Emanuel Besa, whom I had met at an MDS Foundation seminar. Dr. Besa was conducting his trial with a drug called Accutane, which is high in doses of Vitamin E. He had achieved some positive results that showed that Accutane could delay the need for transfusions. So, I started Accutane, which I had to buy from Canada since it was not approved for treating MDS in the US. I took that for about a year while getting CBC tests very often. I then began to need regular transfusions in September 2009. Dr. Besa then referred me to Dr. Erev Tubb at Crozer Hospital.

Dr. Tubb tried me on Procrit, Aranesp, and then both together, but they did not work.

Medication regime

I then started taking Revlimid. As part of the protocol, I had to undergo bone marrow biopsies before treatment began and after it failed. (I’ve had 6 biopsies and found that some doctors are much better at performing them than others)!

Revlimid — 3/25/2010 – 9/12/2010 – Was transfusion-independent for 143 days, then stopped working.

I tried again from 10/25/2010 to 12/14/2010, but there was no response.

Vidaza — 2/14/2011 – 7/19/2011 – Was transfusion independent for 33 days and 42 days, then no response.

And so, after Vidaza failed, I thought I was a goner!

I wish someone had told me that blood transfusions are no big deal. They have kept me going for over 10 years. I’ve never had a bad reaction. I have been able to work full-time, and most of the time, I feel almost normal. The nurses at Crozer were terrific.

My biggest issue with transfusions is that some Doctors don’t want to transfuse until the haemoglobin is under 8 after a CBC exam.

This was the policy at Crozer. On my last visit there in 2011, I did a CBC Blood Count, and my haemoglobin level was 8. They told me to come back in 3 days.

I argued that my haemoglobin would most surely go down in one day, but they would not order the CBC kit. When I returned 3 days later, they agreed to order a transfusion but discovered I had developed an antibody. It then took 3 more days to get the blood, and by the time I was transfused, my haemoglobin was below 7.

Do your research

As you can imagine, I decided that practice was not for me. I consulted the Message Board on the MDS Foundation website and, through that, met Bob Weinberg. Bob also had MDS, diagnosed in his late 40s. He was on the MDS Foundation’s Board of Directors and was very helpful. He changed my life by recommending that I go to Lankenau to see Dr. Cliff Pemberton.

On my first appointment with Dr. Pemberton, I did a CBC Complete Count, and my haemoglobin was in my mid-60s. I was not doing well at all. He immediately ordered 2 units and another a few days later, and I finally felt alive again.

Eight years later, I have many antibodies, but the blood bank always comes through! Exjade (Now Jadenu or Desferasirox) has kept my Ferritin in the low 3,000 range since 8/13/2011. I am fortunate that side effects are minimal—diarrhoea and some dry skin issues.

I continue to consult with the haematologists at the University of Pennsylvania because the MDS Foundation has listed them as a Center of Excellence. They know a lot more about MDS than local haematologists.

Through them, I learned about Luspatercept or Reblozyl as it is now marketed. After waiting two years, I finally got my first shot in late April this year. I was considered a good candidate because I have RARS and the mutated gene SF3B1.

The right treatment

IF LUSPATERCEPT (REBLOZYL) DOESN’T WORK FOR ME, I WILL BE DISAPPOINTED BUT WON’T HESITATE TO GET INVOLVED IN THE NEXT CLINICAL TRIAL. AS LONG AS I HAVE THE HELP AND SUPPORT OF MY FAMILY, DOCTOR AND NURSES, I HAVE BEEN GOOD!
Since then, I have had seven injections. My white count, always a little low on a CBC report, has returned to normal, and my platelets have also increased.

Still waiting on the red count to change.

The side effects for me are stomachache, fatigue, dizziness when standing, diarrhoea, and occasional pains in my arms and legs, but nothing I haven’t been able to handle. The side effects seem to diminish over time.

My current doctor at Main Line Oncology is Zonera Ali, and Dr. Pemberton has retired. They have an excellent practice, and the nurses are great. They hardly ever have to wait and do their best to accommodate my needs.

The ongoing research and the emergence of new drugs encourage me. Newly diagnosed patients now have a better chance of receiving treatments specifically designed for their MDS diagnosis rather than having to guess what might work.

Hope

At present, I am 70 years old. Although being transfusion-dependent for the past 11 years has been inconvenient, I am thankful that the blood has been available. At the beginning of COVID-19, when there was a blood and CBC test staff shortage, I sometimes could only get one unit. That was scary for me — at times, my haemoglobin went down to below 7, and when that happened, my quality of life suffered. I need my husband to drive me to the hospital, and it becomes difficult to walk the distance to the infusion room.

Things are a little better now with the blood shortage. I can care for myself as long as my haemoglobin stays in the 8s, although I often sleep and nap.

So, I was laid off from my full-time job in April, so managing all the doctor’s visits has been less stressful. I can relax now, get to know my neighbours, and see my friends more, although Covid has somewhat limited that.

Final thoughts

One of my biggest concerns about retiring was insurance. I had a great plan where I worked and rarely had to pay anything besides my deductible and co-insurance. I contacted a Medicare Advisor who set me on Medicare in two days. Fortunately, Medicare and the supplemental insurance have paid for the Luspatercept shots, and my only out-of-pocket expense is the annual deductible. That was a big relief since each shot costs around $15,000!

So, today, I’m in a good place, even after having MDS for 14 years! The hardest thing about MDS is the fear of the unknown and the worry that it causes my husband and daughter. I worry it will worsen, but I try not to dwell on that.