Toby the Tap

I had my portacath (port), henceforth ‘Toby the Tap’, ‘Toby’ or ‘Tap’, installed yesterday.  Toby is a Bard Power Port (approx. $60USD+install). Installation is a short procedure, about 30 minutes, using local anesthetic and conscious sedation.  Two incisions are made, one in the chest for the reservoir and another in the neck where the catheter bends toward the heart.   The reservoir is about the size of a quarter, the catheter about the diameter of a cooked spaghetti noodle.

Often people do not remember when coming out of conscious sedation.  This was my third time being sedated and the second of the three that I remember what happened.  Unfortunately there wasn’t much to remember as my face was covered with a sheet.  I asked Doc to allow me to watch but apparently that is a no-go which was extremely disappointing.  So I lay there for thirty or so minutes staring at a blue sheet listening to Doc give instructions and answer questions (sounded like he was teaching someone).   The procedure was mostly painless save for when the catheter was being pushed into my chest, as lot of pressure was put on my neck on the side with severe lymphadenopathy (inflammation of the lymph nodes), which was fairly painful.  Once the Lidocaine wore off, a couple hours after the procedure, the upper incision was moderately painful; however, a bit of opium took care of that (technically Percocet but opium sounds cooler).

Tomorrow I have an appointment with Doc Restrepo and a nurse with experience administering SGN-35.  I will decide then whether or not I am going to join the trial.  I am pretty sure I know which way I will go, but if I spill the beans now I’ll have nothing to write about tomorrow. ;)

Decision Time

Doc Restrepo called me yesterday to let me know Seattle Genetics has opened a clinical trial for their new drug SGN-35 (Brentuximab Vedotin) that I qualify for. This is a phase I trail for evaluating the safety of SGN-35 combined with ABVD for HL treatment.  SGN-35 will be administered at 0.6-1.2 mg/kg with each ABVD treatment.

SGN-35 is an antibody-drug conjugate targeted to CD30 (that’s the bad guy we want to whack).  Results have been published from two SGN-35 trails: one for relapsed and refractory (disease that resists treatment) HL patients, and the other for Anaplastic Large-cell Lymphoma (ALCL, a type of non-Hodgkin’s Lymphoma).  In both trials SGN-35 was used alone not in conjunction with chemotherapy.  

In the HL trial 75% of 102 patients achieved an objective response (complete or partial remission), as assessed by an independent review.  34% of patients achieved full remission.  Tumor reductions were achieved in 94% of patients.  18 patients discontinued this trial due to adverse events. To qualify for this trail patients must have previously received an autologous stem cell transplant (ASCT, that means they collect your own stem cells before a chemotherapy regimen kills your immune system then transplant the collected stem cells to re-grow the bone marrow).  The median number of previous chemotherapy regimens for patients in this trail was 4 (range 1-13).

The ALCL trial included 58 patients.  All patients had previously received front line treatments.  SGN-35 was administered alone once every 21 days by IV (1.8mg/kg).  86% of patients achieved an objective response to the drug.  53% achieved complete remission. The most common adverse events were nausea (38%), peripheral neuropathy (38% - numbing of the fingers and the toes), fatigue (34%), fever (33%) and diarrhea (29%).

If I decide to join the trail I will have to get several blood test and scans (CT and PET).  This will delay the start of treatment by about two weeks.  Doc Restrepo feels that given the positive response to the Prednisone and Cyclophosphamide I was on delaying treatment will not adversely affect prognosis. While taking the drugs all of my b-symptoms were relieved but since completing the cycle I have experienced some fatigue and discomfort in my neck.  Both are very minor compared to before the drugs but I am concerned that will change inside of three weeks.  I cannot take the Prednisone or Cyclophosphamide in the interim due to the side effects, and as a result I risk the disease progressing in the interim. 

Re-staging

I received a call from my Oncologist today to tell me that a committee, after reviewing my CT scans, has decided that I have stage IV HL not stage III.  This is due to the pericardial effusion and masses in my lungs.  The committee still believes the original treatment plan is the best course of action so I will start six cycles of ABVD on April 1^st 2011.

My Hasenclever Hodgkin’s Prognosis Score (IPS) is 3.  This means I have a predicted 5-year freedom from progression of disease of 60% and overall survival rate of 78%.  The Hasenclever system was developed in 1998 using data from patients who were treated with ABVD or MOPP chemo regimens.  The risk factors in the Hasenclever system which are present in my case are: sex (male), stage IV disease and white blood count ≥ 15,000/µl. When I had my first CBC in January 2011 my WBC was below the threshold to be considered a risk factor based on Hasenclever. 

Other studies have shown the most important adverse prognostic factors are: mixed-cellularity or lymphocyte-depleted histologies [no], male sex [yes], large number of involved nodal sites [yes], advanced stage [yes], age of 40 years or more [no], the presence of B symptoms [yes], high erythrocyte sedimentation rate (results not in yet), and bulky disease (widening of the mediastinum by more than one third, or the presence of a nodal mass measuring more than 10 cm in any dimension) [no].

Drugs, Drugs, Drugs

I have received my pre-chemo and chemo support drugs.  Between these drugs and the drugs I received during diagnosis I have a pretty nice pharmacy at home. ;)

As mentioned in my last post I was given Prednisone and Cyclophosphamide (100mg each per day for several days) to ease the inflammation and symptoms of HL.  These have done a magnificent job. Within two days of starting the drugs the lymphadenopathy in my neck has reduced greatly and I am no longer suffering from fatigue, night sweats or fevers.  

The only side effect I have noticed thus far (I am on day six of seven) is an insatiable appetite (common side effect of corticosteroids, in this case Prednisone).   From three to four days after starting the Prednisone I have been unable to satiate my appetite no matter how much I eat or how full my stomach is.  No matter what, I always feel like eating.  Yesterday, after eating far too much food during the day, I went to Players Chop House for dinner.  Apparently the largest prime rib on menu, the 16oz Player size, was not big enough for me.  I ordered the “Texas Player Size” (I named it that after telling the waitress I was from Texas). 

The meal was so large that every server in the restaurant came by to examine my progress and note their amazement.  I ate the entire plate of food, plus half a dish of additional mushrooms, plus half of a dessert.  I also drank two beers.  This is after eating a considerable amount during the day leading up to the event. 

My chemo protocol will consist of three support drugs and the ABVD chemo regimen.

Chemo Regimen:

Adriamycin - Comes from a fungus which is found in soil. It works by stopping DNA replication. It is also known as Doxorubicin or The Red Devil due to its colour.  Side effects of Adriamycin include: nausea, vomiting, heart arrhythmias, neutropenia (decrease in while blood cells) and alopecia (hair loss).  Once dosing reaches 550 mg/m², Adriamycin can be cardiotoxic.

Bleomycin - Also from a soil fungus. This one acts by breaking up DNA.  The most serious side effect of Bleomycin is pulmonary fibrosis (hardening of the lungs) leading to permanent lung impairment.   This should be avoidable by monitoring pulmonary function and correct dosing. Other side effects include: fever, rash dermatographism, hyperpigmentation, alopecia and Raynaud’s phenomenon (discoloration of the fingers and toes).

Vinblastine - Interferes with cell mitosis (mitosis is half of the process of cell division). It is a vinca alkaloid similar to Vincristine but with fewer side effects. It comes from a Periwinkle plant native to Madagascar.  Common side effects of Vinblastine: bone pain, constipation, depression, diarrhea, general body discomfort, headache, jaw pain, loss of appetite, nausea, stomach pain, and vomiting.

Dacarbazine - Breaks up DNA and RNA and prevents their synthesis.  Dacarbaize can be painful when given intravenously.  Often it is diluted with saline to ease the pain.  If the needle is not in the vein correctly it can also cause muscle/nerve damage.  This should not be an issue for me as I will receive my drugs via a port.  Side effects of Dacarbazine: possible permanent sterility (mitigated through prior deposits), immune suppression, nausea, headache and fatigue.

The combination of drugs increases the likelihood of developing Acute Myleoid Leukemia 5-7 years after treatment.  Short term or permanent peripheral neuropathy (numbing of the fingers/toes) is also a risk with the regimen.

Support Drugs:

Ondansetron (pre-chemo) – Is used mainly and an antiemetic to treat nausea and vomiting from chemotherapy.  It has little effect on vomiting and nausea caused by motion sickness.  Ondansetron is a well-tolerated drug with few side effects. Constipation, dizziness and headache are the most commonly reported side effects associated with its use.

Dexamethasone (pre-chemo and two days following) – Is a strong corticosteroid 4-5x more powerful than Prednisone.  It is anti-inflammatory and immunosuppressant.  Its primary use for me will be to augment the effects of Ondansetron.  Apparently Dex is popular among Bangladeshi prostitutes because it helps them gain fat easily, which is attractive in the poor country.   Most common side effects: weight gain due to increased appetite, immunosuppression, psychiatric disturbances, including personality changes, irritability, euphoria, and mania.  Withdrawal is common issue with Dex.

Metoclopramide (As needed) - Is an antiemetic and gastroprokinetic agent (it enhances gastrointestinal motility).  Common side effects include: restlessness, drowsiness, dizziness, lassitude, and/or dystonia.

I have discussed switching the Dexamethasone with Aprepitant.  My Oncologist would like to wait until we see how infusion 1A goes before deciding to switch, as Dex is the standard given by the BCCA.  Aprepitant has far fewer side effects than Dex but is very expensive; however, given my medical coverage this will likely not be an issue.

Shrinkage and Meat Slam Pt. 1

I’ve been taking Prednisone and Cyclophosphamide for two days now and they have already made a significant impact on the lymphadenopathy in my neck. The supraclavicular lymph node that had grown to ~7cm and was causing me significant discomfort is now about 4.5cm and painless. In addition I haven’t had a fever since starting the drugs although the night sweats persist.

My first chemo has moved due to a conflict with my port install. I will now receive my port on March 29th and chemo 1A on April 1st. A port or portcath is a medical appliance installed beneath the skin that connects a catheter to a vein. This will allow me to receive IV drugs and have blood drawn without using veins in my arm. The benefit of using a port is prevention of damage to veins and eliminating the risk of drug leakage into muscles (leakage can cause permanent muscle damage).

 

During chemo I will not be permitted to eat raw fish or raw meat due to risk of infection.  Six months without sushi or rare beef requires some preparation; I started this today at The Keg.

16oz of sexy:

Staging and Treatment Plan

I saw my Oncologist for the first time today.  I am being treated by Dr. Diego Villa Restrepo at the BCCA.  Yeah, his name is Restrepo.  Go see the movie.  It's good. 

The CT results of my abdomen and pelvis showed involvement below the diaphragm so I have been officially staged as IIIb.

CT Abdomen and Pelvis Report

History

Work up Lymphoma

Technique

Contrast enhanced images were obtained through the abdomen and pelvis.

Findings

There is a moderate-sized pericardial effusion identified measuring up to 1.6cm in thickness.  There are enlarged lymph nodes up to 15mm short axis anterior to the esophagus near the abdomen hiatus. Enlarged lymph nodes are also present in the gastrohepatic ligament and cellac axis region measuring up to 2.1cm short axis.  Enlarged paracaval lymph nodes are seen from the level of the SMA distally.  These measure up to 18mm. From the level of the aortic bifurcation distally there are small lymph nodes present but none are markedly enlarged. A right lliac chain lymph node measures 10mm short axis.  The spleen measures up to 15.9cm and contains three round low-attenuation lesions measuring up to 14mm.  These do not appear cystic.  No focal hepatic lesions are seen and the gallbladder, adrenals and pancreas are within normal limits.  No focal renal abnormalities are seen.  There is a trace of free fluid in the pelvis.  The appendix is unremarkable.

Impression

Pericardial effusion. Splenomegaly with associated focal splenic lesions which can be seen in lymphoma.  Mild intra-abdominal and pelvic lymphadenopathy which is more prominent in the epigastric region.

Treatment will be 6 cycles of ABVD chemotherapy.  Each cycle of ABVD is 28 days with an infusion on the 1^st and 15^th day.  Post chemo I will receive a CT and possibly PET scan to determine whether or not radiation will be necessary.   I will post more details about ABVD later this week.

Pre-chemo I will have an echocardiogram to get a baseline heart function to compare against during treatment should heart issues arise.  The A drug in ABVD, Adriamycin, can be cardiotoxic, which means it can be toxic to the heart. 

I have been prescribed Prednisone (100mg every morning for 7 days) and Cyclophosphamide (100mg every morning for 7 days) to ease the inflammation and increase comfort while waiting for chemo to start. 

ABVD will likely begin on March 29^th.

The beginning

On March 10^th 2011 I was officially diagnosed with Nodular Sclerosing  Hodgkin’s Lymphoma.  I have created this blog to keep those interested up to date on what is happening with my treatment and to hopefully provide some insight for others diagnosed with HL on what to expect during treatment.

In early Jan 2011 I visited my GP about a small lump in my neck.  At the time I had no other symptoms.   After examination of the lump he ordered blood work, which revealed I was slightly anemic.

Blood Results:

	Flag	Results	Reference Range	Units
Hematology
WBC	H	11.5	4.0 - 11.0	10*9/L
RBC		4.69	4.30 - 5.90	10*12/L
Hemoglobin	L	115	135 - 180	g/L
Hematocrit	L	0.37	0.41 - 0.52	L/L
MCV	L	78	80 - 100	fL
MCH	L	24.5	27.0 - 34.0	pg
MCHC	L	314	323 - 365	g/L
Platelet Count		384	150 - 400	10*9/L
Differential
Neutrophils	H	8.8	2.0 - 8.0	10*9/L
Lymphocytes		1.7	1.0 - 4.0	10*9/L
Monocytes		0.7	< 0.9	10*9/L
Eosinophils		0.2	< 0.8	10*9/L
Basophils		<0.1		10*9/L

Upon receiving the blood results he performed a physical, ordered more blood work, a urine test, and a chest x-ray.  Shortly after this appointment I started experiencing many typical HL symptoms.  Several other lymph nodes in my neck became inflamed to the point that they were visible.  They did not hurt but there was discomfort when I moved my neck.  I also experienced discomfort in my chest, difficulty breathing at times, mild fatigue, night sweats, increased resting heart rate at times, and skin irritation.

Chest X-Ray Report

Widening of the right side of the mediastinum is noted consistent with right paratracheal and right azygos lymphadenopathy. Slightly enlarged bilateral hilar lymph nodes cannot be excluded. Subcarinal lymphadenopathy cannot be excluded either. A small amount of fluid is present in the right-sided fissures. Peribronchial thickening is present in the right lower lobe. Minimal patchy consolidation is also present in the right lower lobe. The heart and left lung are normal in appearance.

Impression

Right-sided mediastinal lymphadenopathy. Bilateral hilar and subcarinal lymphadenopathy cannot be excluded.

Right lower lobe bronchopneumonia. Small right pleural effusion.

The findings are consistent with Hodgkin's disease. Sarcoidosis cannot be excluded although the small right pleural effusion is unusual.

Surgical consultation and CT scan of the chest is recommended.

As you see from the report, the chest x-ray was the first indication I had HL.  I was referred to a Pulmonologist who performed a fiberoptic bronchoscopy with biopsy; I also had a CT scan.   Pathology of the sample retrieved from my lung during the bronchoscopy showed no evidence of Lymphoma or Sarcoidosis. 

CT Scan report:

CT Neck and chest enhanced

History

Severe clavicular and mediastinal lymphadenopathy, sarcoidosis or Hodgkin’s disease.

Comparison

No prior studies available for comparison

Findings

Extensive lymphadenopathy throughout the next measuring up to 2.1 cm in short axis diameter.  Extensive lymphadenopathy in both supraclavicular regions.  No other soft tissue abnormalities in the neck.

Extensive mediastinal lymphadenopathy.  A large heterogeneous anterior mediastinal mass measuring 6.1 x 3.8 cm in axil diameter in most likely confluent lymphadenopathy. Lymphadenopathy also involves the hilar regions and subcarinal region.  No axillary lymphadenopathy.  No pericardial effusion.  The cardiac chambers are normal in size and appearance.

At least six small non calcified pulmonary nodules are scattered in the right lung, most of which are either subpleural, or along the interlobar fissures.  No definite pulmonary nodules in the left lung.  The trachea and central bronchi are patent and normal in caliber.

Limited images of the upper abdomen are unremarkable.  No suspicious destructive osseious lesions in the neck and chest.  The overlying soft tissues are normal.

Impression

Extensive lymphadenopathy in the neck, supraclavicular regions and throughout the mediastinum with a large confluent nodal mass in the anterior mediastinum.  The differential diagnosis includes sarcoidosis or lymphoma, but the presence of scattered non calcified pulmonary nodules, most of which are subpleural or along the fissures favours diagnosis of sarcoidosis.

The Pulmonologist referred me to a surgeon to have a larger sample taken from my neck.  The surgeon disagreed with the CT doctor's findings he felt sarcoidosis was very unlikely.   To confirm diagnosis of HL an excisional biopsy of an infected lymph node must be performed, as a needle biopsy will not provide a sufficient sample.  The surgeon took a 1.6cmx1cmx0.6cm sample from the right side supraclavicular lymph node. This was the first to become enlarged and is by far the largest node.

Scar the morning after biopsy

       

 Scar about a week later:

In addition to the lymph node biopsy a bone marrow biopsy was performed to determine whether or not the cancer had spread to the bone marrow. Bone Marrow involvement would mean stage IV disease which has a much poorer prognosis than stage I-III, which are all quite similar.

The bone marrow biopsy came back clean.   Results from the CT of my abdomen and groin are not in yet so we do not know if it is stage IIb or IIIb disease.   Stage II indicates that more than one lymphatic region is affected (in my case three, both sides of the neck and my chest) on the same side of the diaphragm.  The ‘b’ indicates I exhibit systemic symptoms. Stage III indicates lymph node involvement on each side of the diaphragm.

Diagnosis has taken about 10 weeks.  In that time my right supraclavicular lymph node has grown from ~2cm long to ~7cm in length.  During the past 3 weeks I’ve been having low-grade fevers almost daily (200mg of Ibuprofen has helped A LOT) and the fatigue has become much worse.

Prognosis for HL is very good, with recovery at over 80% for my stage and age. I will likely receive six months of ABVD chemotherapy, possibly followed by radiation therapy.

---