Cancer was not part of the plan

22.02.2021
Patient stories
Annual Report 2020

The domino effect – secondary immune deficiency

On top of CLL, Ingrid was also diagnosed with secondary antibody deficiency, a type of secondary immunodeficiency (SID). SID is a common complication in patients with haematological malignancies such as CLL.

Read Ingrid’s story below

Ingrid has always enjoyed life. “I laugh a lot. I have fun,” she says. “I’m content. Most of the time I’m calm and relaxed. This disease, this cancer, was not part of the plan I had for my life – but there it is.”

In March 2003, Ingrid, who lives with her husband in a small town in Hesse, Germany, was diagnosed with chronic lymphocytic leukaemia (CLL) – just nine years after her sister died of acute leukaemia. “What was meant to be a reassuring check-up with my doctor turned my life upside down”, she remembers. Ingrid had a small lump in her groin and was given a precautionary blood test which revealed an increased number of leucocytes – white blood cells – the first sign that she had CLL. The first weeks after her diagnosis are still very clear in her mind. “Honestly, it was awful. I thought of my sister, of course, and was sure I was soon going to die”, she says, candidly.

Fortunately, Ingrid’s initial fears did not come true and, immediately after her diagnosis, at the age of 51, she began to ‘watch and wait’ – a common approach to CLL in which patient and doctor monitor the condition without treatment. Eventually, however, after five years of active surveillance, in 2008 a change in Ingrid’s blood values persuaded her doctor to initiate a therapy..

CLL is a cancer of the immune system. It starts in the body’s infection-fighting white blood cells, called B cells. As it progresses, it produces abnormal white blood cells in the bone marrow and blood that cannot fight infection. Because CLL is a slow-growing cancer, some people do not need to start treatment for many years – as was the case for Ingrid.

Ingrid’s doctor laid out a couple of options which would hopefully kick the CLL into remission for an indefinite period. However, when her symptoms eventually reappeared a few years after the first therapy, Ingrid went through further treatment cycles in 2013 and 2019.

CLL explained

What is chronic lymphocytic leukaemia ?

CLL is a type of blood cancer that starts in the bone marrow and affects the lymphocytes, which are a type of white blood cell. The cancerous CLL cells grow slowly, displace the healthy blood cells, then propagate in the bone marrow and blood stream.

In patients with CLL, the immune system does not function normally, and one of the most common complications is frequent infections.

Read more on secondaryimmunodeficiency.com

The domino effect

On top of everything else, Ingrid was also diagnosed with secondary antibody deficiency, a type of secondary immunodeficiency (SID). With SID, the immune system is weakened and patients become more susceptible to infections, increasing their morbidity and mortality. SID is a common complication in patients with haematological malignancies such as CLL, with up to 85% of CLL patients developing antibody deficiency during the course of the disease, either due to the underlying disease or as a side effect of their treatment.

For more information on SID and haematological malignancies, you can visit the website: secondaryimmunodeficiency.com.

Patients like Ingrid are given immunoglobulins (either intravenously in a clinic or subcutaneously by self-administering at home) to boost their low levels of antibodies to help to protect them against infections. Ingrid started her treatment with immunoglobulin in November 2015, initially with intravenous infusions (IVIg) but this was recently switched to Octapharma’s subcutaneous human normal immunoglobulin treatment (SCIg), to keep her antibodies more in balance. “With immunoglobulin therapy, I am less afraid of developing infections,” says Ingrid.

“Compared with IVIg, I now have more stable immunoglobulin G (IgG) levels with SCIg, which seems to be more effective,” she continues, adding: “During the COVID-19 pandemic, it is even better to use SCIg as I don’t need to go to hospital.”

Ingrid infuses at home by injecting purified immunoglobulin into the fatty tissue just underneath the skin. “Honestly, I’m afraid of COVID-19, so infusing from home is much safer for me. I don’t want to die lying in an intensive care unit on a ventilator.”

“I am so lucky to have found myself surrounded by such support,” says Ingrid.

Sailing through uncharted waters

Over the years since her diagnosis and during her treatments, she has sometimes been numb, or beyond sad, and at other times has felt optimistic. But what has always kept her fighting is her family, her children and grandchildren, whom she is most proud of in life.

She acknowledges that the love of her entire family, friends, and even her dear dog has, over the years, been central to making everything more bearable. “I am so lucky to have found myself surrounded by such support, to have felt so loved and cared for by so many people,” says Ingrid. “All of them have really helped me in their own way when I needed it, allowing me to stay as focused as I can on trying to figure out what the next step should be, as I sail through these uncharted waters.”

Weeks, even days, can still be full of highs and lows, but as she puts it: “It’s okay to be anxious and scared about this. But remember that you won’t have those feelings 24 hours every day and that there are many great things to do in life. Just focus on that, stay positive and try to stay calm despite your disease.””

Treatment

Standards treatments include:

  • Radiation therapy

  • Chemotherapy

  • Surgery

  • Targeted therapy

  • Chemotherapy with stem cell transplant

  • Immunotherapy

Octapharma working to support CLL patient care

Octapharma recently launched a phase III clinical trial in patients with CLL and SID. The PRO-SID trial is the first randomised, placebo-controlled study to systematically evaluate the efficacy and safety of immunoglobulins (IVIg) for primary prophylaxis for infection control in patients with CLL.

“Despite and even because of improved B-cell targeting therapy options in CLL patients, infections are still one of the major causes of morbidity and mortality”, says Wei Ding, MBBS, PhD, Mayo Foundation for Medical Education and Research, who sits on the steering committee of the PRO-SID study. “Primary prophylaxis with IVIg before severe infection occurs has the potential to reduce infection rates and the burden on patients and the healthcare system.”

Dr Olaf Walter, Board Member at Octapharma, adds:

“There remains a significant need to reduce the burden of the disease in managing patients with haematological malignancies and secondary immunodeficiency. Initiation of the PRO-SID study represents a key milestone in Octapharma’s efforts to improve the care of patients with CLL.”

Keywords

Annual report

Diseases & therapies

Immunology