Warning: images and text may be disturbing
This is my personal story, and not intended to be medical advice. Turn away if you wish.
And He Said
And he said, “I will create another disease.” And he did.
The Rear View Mirror
I am a normally healthy 72 year old female. Throughout my sixties could rice 80 miles a day on my bicycle, dance 3 times a week and canoe for weeks in the wilderness of Quetico Provincial Park in Ontario.
I had IBS, mild arthritis, but life was good and I was active.
I have always been like a kid, who, after school, can’t wait to go out and play. I am all about having fun, being outside and being active, and doing stuff with family and friends. I’m very social.
Were there warning signs of Sezary Syndrome? About three years ago I started getting Dandruff. In the months before diagnosis I had mouth ulcers and perianal cysts. Was there a connection? Was that a clue? No one knows.
I must have had Sezary well before the diagnosis. Sezary developed over time. A diagnosis is a point in time. How long did I have Sezary before I was diagnosed? Months, years, Who knows? It couldn’t have appeared overnight.
The Dermatologist
I called repeatedly. My skin was driving me crazy. I was put off, deflected. When I finally got into the office they did 2 biopsies and sent me to the emergency room. That was a good move, because tests starting being done one after the other, and results poured in. Still no definitive diagnosis, but Sesary was mentioned. I had heard of it from a doctor friend, had looked up the symptoms, and didn’t think that could be what I had. The team in the hospital wasn’t sure either, but it was on the list, along with mycosis fungoides. One of the tests done was a flow cytometry.
The Asshole
Two weeks later I had an appointment with a new dermatologist. He was vary casual about telling me I had Sezary Syndrome. It was a gut blow. WHAT?!!!
What is the life expectancy? Five years on average.
He said five years was better than nothing, in a casual and dismissive way. I asked how he knew. He said he knew all about sezary, because, “I am a Sezary expert and on all the medical board that focus in Sezary.” I think he was please to have a local case. Less than one in a million people have Sezary. And besides, the flow cytometry test found 35% Sezary cells in your blood. So there!
I wanted to strangle him. “
My New Life
I keep a journal. and more recently a consolidated time line on a spread sheet. I’ll share that with you later. I am trying to figure out cause and effect. Is there a pattern? Is this treatment, ointment cream or lotion working? I have a box full of prescription and over the counter lotions and creams. And lots of pills too.
The approach is bifurcated. 1. Kill the cancer 2. Get relief from the symptoms.
1. Kill the Cancer
This is a short section, because first of all, you can’t kill it. You can only slow it down. Secondly, thus far we have had only one bit of good news, and it just arrived, 12 months after diagnosis. In my first Flow cytometry test I had 35% sezary cells in my blood The Sezary cell count just dropped from 35% to 5%, AND We think it is because of the Mogomulizamab. That was the first treatment my oncologist tried. However it was briefly interrupted after 2 months for a shot at Brentuximab for one week and then a switch to Pembrolizumab for one week and them back on Moga. We dropped the Brentux and Pembro because of side efficts. The Brent gave me neuropathy while I was still in the charir with the IV in my arm. We stopped the Pembro because we thought it caused neuropathy. After those two flip flops I went to the MAYO Clinic in Phonenix for a consult and then went back on Moga.
So the conclusion is, the drop from 35 to 5% is most likely due to Moga (or a mericle – a friend did do a new age spiritual thing and maybe it worked. I like science but I keep my options open.)
FYI All treatments aimed at killing cancer have been administered IV.
2. Get Relief From The Symptoms
This is a long story of trial and error, and it does not cure cancer, it only helps you to endure.
Symptoms:
Red Skin, Poor temperature control (chills and fever), Severe itching, Flaking and pealing skin, Hair loss, Lesions, Enlarged lymph noads, depression, anxiety, neuropathy, possible infection (sepsis)
The first 6 months after diagnosis were the worst. My discomfort cycled between bad and really bad. I was so uncomfortable that I became nonfunctional.
A Poem
Similes, Metaphors, and Questions
Sezary is a battle, like war, or a game of tennis, or basketball, or football. But your opponent has more weapons and tools than you. He can throw faster, jump higher, hit harder, and knows the rules better than you. You might make a good play, but he is right back at you with a change in strategy, renewed vigor, patience, and persistence.
Sezary is like Wack a Mole. Wack one symptom and another pops up somewhere else.
Is sezary a test, or a punishment. Think Jobe or Sisyphus.
Time Line
Early symptoms to diagnosis
April to June 2023. Life was normal. I am retired. I am a potter and was able to create in my studio. I traveled to dance event on weekends. I did my birthday bike ride. It used to be 100 miles, but now I ride my age. This year it was 71 miles. I had IBS but coped, and some arthritis too. But I considered myself a fairly healthy and fit person, and mentally I said I was 35 years old. I have very low cholesterol. I take metropolol for irregualar heart rhythm.
Between June and August 2023 things started happening that may have been early symptoms of Sezary, but no-one even considered Sezary. I started experiencing some itching on my hands and belly. OK, maybe that’s eczema, or hives? And perianal cysts, and mouth ulcers. No-one had a clue, but they did punch biopsies. Nothing pointed to Sezary, but they weren’t looking for it either. Not looking at T cells.
Itching persisted and redness appeared. I became more and more miserable. I describe my condition as “like a bad sunburn with poison ivy on top.” My skin was super itchy and everything I touched was an irritant. It got worse. Every seam on my clothes was an irritant. Even soft cotton was irritating. Had I been suspended in a zero gravity bubble I would still have been miserable. My quality of life dropped to zero! I couldn’t function. Everything I touch was painful. My dermatologist was too busy to see me. I banged on the door but couldn’t get seen. Finally September 8 I had an appointment. They were shocked. Two more punch biopsies were done. It was suggested I go to the emergency room so I could gets more tests done quickly. So I did. They appologized for not seeing me sooner.
September 8-15-2003 In hospital. Prednisone slowly releaved symptom. Sezary was mentioned but not seen likely, discounted.
The first 8 months after diagnosis
I need to write more here. Things do improve somewhat. lorem ipsum dolor sit amet, consectetur adipiscing elit. Ut elit tellus, luctus nec ullamcorper mattis, pulvinar dapibus leo.
Daily Log
In addition to notebook, we keep a log on an iPad in a database. It took a while to refine what symptoms we should track. Itching, peeling, dry skin, and redness were obvious categories. But flaky itching scalp and tiredness were reverent too. And so was weight loss. QL refers to “Quality of Life,”, where 1 horrible, and 10 is able to function almost like a normal before Sezary. We never have a 10, but we do have 9’s. In the notes we usually mention whatever happened that day in terms of activities, etc., and also make note of any relevant Sezary issues.
Treatment Summary
Actual Medication to Kill Sezary
Talk about moga etc in this section
Creams, Ointments and Lotions
Killing Cancer Cells
None of these kill cancer, but they do relieve the skin symptoms and can help reduce the chance of infection.
Medicated stuff: Clobatasol for intense itchy spots. Triamcinolone for larger less itchy spots, Cortisone 1 or 2% for even larger less itchy areas. Triple Antibiotic Ointment on minor scratches to prevent infection. These were prescribed early on and are still used as needed. They all have damaging side effects so I use them judiciously, only as needed.
I take 50mg of hydroxyzine four times daily.
When my condition is sever I take up to 40mg of prednisone a day. It shut down kidney function so I either taper off or don’t take it for more than 6 days without a break.
Next Group: CeraVe Moisturing Cream comfort, Aquafore for Comfort, Coconut oil and other assorted massage oils for massage and comfort. I can use as much as I want.
Massage: There was a time early on where I couldn’t be touched because my skin burned and itched so badly, and was so sensitive. Would you want a massage if you had poison ivy, or a sunburn?
Every ruff edge hurt me. I couldn’t open a package or a box, or unscrew the lid of a mayonnaise jar. I still can’t unscrew the lids. But now I am feeling better and get relief from massage. I loved massage before Sezary. Now that I’m doing better and it feels good again.
Next Group: Baths and wet wraps. These can provide relief and they keep shin bacterial growth down. This is important because your skin is mess. We are normally covered with all lkinds of bad bacteria like staff, but our body keeps it under control. With Sezary our skin barrier is compromised and so a small scratch can lead to a deadly infection. So these baths help control bacteria on your skin.
Vinegar Bath Wet Wrap: You can look this up. I did not like it but it works for some people.
Bleach Bath: half a cup of Clorox in a bath tub with 8 inches of water. This seemed to help reduce peeling although not redness so much. It definitely kills bad bacteria and that helps prevent infection and seems to promote healing of cracked flaky skin. I was resistant to bleach baths at first because they seemed like they would be too drying. If I do them twice a week that works for me. I soak for 15 minutes in the tub and then shower off and apply creams promptly. It seems to work.
Need to write more here.
Lorem ipsum dolor sit amet, consectetur adipiscing elit. Ut elit tellus, luctus nec ullamcorper mattis, pulvinar dapibus leo.
On Death and Dying
Chat GPT quires and responses in this shaded area
Well, I’m not dead yet so I can give you a full report at this moment. Here is what I can say thus far.
I love life. I am a very fun loving person, and an outdoors person, and very social. I care about people. I don’t want to say goodby.
That said we must all die, but this is not the death I thought I would have. And what about death by Sezary. What will it be like. Ugh!
I have heard you don’t die from Sezary, you die with Sezary. I guess that means it wont kill you directly, but it will hasten your death, and some other organ besides you skin (FYI your skin is your largest organ) will do you in, or maybe an infection will get you, like MRSA or sepsis.
Life expectancy is five years on average. So some people last only one year, others for 20 years or more. Like many things, we all react differently. Our journeys are unique.
During low points, when my quality of life is zero, suicide seems like a good option. Close the door, “shuffle off this mortal coil.”
So what is known. Let’s get some facts from Chat GPT.
QUESTION
What is the life expectancy for people with Sezary syndrome and what are the causes of death?
ChatGPT
Sezary syndrome is an aggressive form of cutaneous T-cell lymphoma (CTCL), characterized by the presence of cancerous T-cells in the skin, blood, and often the lymph nodes. It is a relatively rare condition and is more commonly diagnosed in older adults.
Life Expectancy
The life expectancy for individuals diagnosed with Sezary syndrome can vary widely, depending on factors such as the stage of the disease at diagnosis, the patient’s overall health, response to treatment, and presence of any complications. Median survival times reported in the literature generally range from 2 to 8 years after diagnosis. Early-stage disease may have a better prognosis, while advanced stages are associated with shorter survival.
Factors Affecting Prognosis
- Stage of the Disease: More advanced disease at diagnosis typically correlates with a poorer prognosis.
- Age and Overall Health: Older age and poorer general health can negatively impact survival.
- Response to Treatment: Those who respond well to initial treatment may experience longer survival times.
Treatment Approaches
Treatment typically involves a combination of skin-directed therapies (like topical corticosteroids or phototherapy) and systemic treatments. Systemic treatments may include retinoids, interferon, systemic chemotherapy, and new targeted therapies like mogamulizumab, which are designed to target specific aspects of the T-cells or the immune system involved in the disease.
What are the causes of death?
The causes of death in patients with Sezary syndrome typically relate to:
- Complications from the Disease: These might include severe infections due to compromised skin integrity and immune dysfunction.
- Progression of the Disease: Widespread skin involvement and tumor formation can lead to significant physical complications.
- Treatment Complications: Treatments, especially aggressive chemotherapy, can lead to side effects such as severe infections, organ damage, or failure.
- Secondary Cancers: There is an increased risk of developing other cancers.
The exact course of the disease can vary significantly, so individual prognosis and experiences can differ. Regular follow-ups with healthcare providers who specialize in dermatology or oncology are crucial for managing Sezary syndrome effectively.
NEW QUESTION
NEW QUESTION
I want to know more about treatment approaches to Sezary syndrome, what they are, how the work and their efficacy?
ChatGPT response
Sezary syndrome, being a complex and severe form of cutaneous T-cell lymphoma (CTCL), requires a multi-faceted treatment approach that may involve skin-directed therapies, systemic treatments, and sometimes more aggressive options like stem cell transplantation. Here’s an overview of the key treatment modalities, how they work, and their efficacy:
Skin-Directed Therapies
- Topical Corticosteroids: These are applied directly to the skin to reduce inflammation and kill cancer cells on the skin’s surface. They are more effective in early stages or for less severe manifestations.
- Topical Chemotherapy: Drugs like mechlorethamine gel are applied to the skin to directly kill cancer cells.
- Phototherapy (UVB or PUVA): This involves exposure to ultraviolet light, which helps to kill the cancer cells in the skin. PUVA therapy combines UVA light exposure with a drug called psoralen, which makes the skin more sensitive to the light.
Systemic Treatments
- Retinoids (such as bexarotene): These are vitamin A derivatives that can help slow the growth of cancer cells.
- Interferon alpha: This is a type of immune system protein used as an injectable form to boost the immune system’s ability to fight cancer.
- Chemotherapy: Drugs like gemcitabine or liposomal doxorubicin are used to target and kill rapidly dividing cells, including cancer cells. Systemic chemotherapy can be more effective in advanced cases but also comes with more severe side effects.
- Histone deacetylase inhibitors (HDAC inhibitors such as vorinostat): These drugs work by interfering with the DNA in cancer cells, causing them to die.
Targeted Therapies and Immune Therapies
- Mogamulizumab: A monoclonal antibody targeting CCR4 (a receptor found on the surface of Sezary cells), helping the immune system recognize and destroy cancer cells.
- Alemtuzumab: Another monoclonal antibody that targets CD52, a protein present on the surface of T cells and some B cells, leading to their destruction.
- Brentuximab vedotin: Targets CD30, a protein that may be expressed on the surface of Sezary cells.
Experimental and Emerging Therapies
- New targeted therapies: These include drugs that target specific molecular pathways in Sezary cells.
- Immune checkpoint inhibitors: These are drugs designed to help the immune system recognize and attack cancer cells more effectively.
- CAR T-cell therapy: Although in its experimental phases for CTCL, this therapy involves re-engineering T cells to better fight cancer.
Efficacy of Treatments
- Skin-directed therapies are generally effective for symptoms and lesions confined to the skin but do not address systemic disease.
- Systemic therapies like interferons and retinoids have variable efficacy but are crucial for controlling more widespread or advanced disease.
- Mogamulizumab has shown promising results in improving survival and quality of life, particularly for patients who have failed other therapies.
- Chemotherapy can be effective but often as a palliative approach in very advanced stages due to its systemic toxicity.
Treatment Challenges and Considerations
The choice of treatment depends on numerous factors, including the extent of the disease, symptoms, patient’s overall health, and previous treatments’ efficacy and tolerance. Due to the aggressive nature of Sezary syndrome, treatments may need to be aggressive, and managing side effects is a critical aspect of care. Close monitoring and regular adjustments in treatment strategies are necessary to manage this challenging condition effectively.
NEW QUESTION (Still Chat GPT)
What is the life mean, median, and mode of life expectancy for people with sezary syndrome?
When discussing the life expectancy of people with Sezary syndrome, it’s helpful to understand the different statistical measures:
1. Median Life Expectancy
- Median is the middle value in a set of data, meaning half of the patients live longer than this time, and half live less.
- For Sezary syndrome, the median life expectancy is typically reported to be 2 to 4 years from the time of diagnosis. This is often the most commonly cited figure in clinical settings.
2. Mean (Average) Life Expectancy
- Mean or average life expectancy is calculated by summing all individual life expectancies and dividing by the number of individuals.
- The mean life expectancy for Sezary syndrome can vary slightly from the median, but it’s generally in the same range, around 2 to 4 years. However, because the mean can be influenced by outliers (patients who live much longer or shorter than expected), it might not always represent the typical experience as accurately as the median.
3. Mode Life Expectancy
- Mode is the most frequently occurring value in a dataset.
- There isn’t specific data readily available that identifies a mode for life expectancy in Sezary syndrome, but it would likely be close to the median, within the 2 to 4 years range, assuming this is the most common survival time for patients.
Summary
- Median Life Expectancy: 2 to 4 years
- Mean (Average) Life Expectancy: Approximately 2 to 4 years
- Mode Life Expectancy: Likely 2 to 4 years, though specific mode data is not commonly provided
These measures give a general understanding of the life expectancy, with the median being the most commonly referenced statistic in clinical practice.