Fibrinogen Amyloidosis Patient Timelines

This page will be used to document the major milestones of each person's journey with fibrinogen amyloidosis. In addition to being a convenient place to record this information and update it over time, hopefully this page can be of benefit to anyone who stumbles upon it so they can see some examples of the progression of this disease.

I'm not limiting this page to members of my family, so if you have the fibrinogen mutation and want some of your information included here so that it may benefit others in the future, please send me an email (toe at juno dot com). I'll identify you however you wish, whether that's full name, first name only, initials, a pseudonym, a number, or whatever.

(This page initially created November 17, 2012)

Kindred #1

Linda J. (July 1941 - October 2015) (daughter of Marshall M.)
(Three offspring: One positive (David J.), one negative, one untested.)
(Two siblings: Both untested. One deceased.)
(Added to page November 17, 2012)

2008 - 2009: Seeing a nephrologist to determine cause of proteinuria and elevated creatinine level.

2010 January: Kidney biopsy shows presence of amyloidosis, but unable to positively determine type.

2010 March 11: Bone marrow biopsy shows no sign of amyloidosis.

2010 June: Evaluated at Boston University Amyloidosis Center. AL and AA amyloidosis ruled out. Familial suspected, but not ATTR.

  Creatinine=1.98; GFR=25; Proteinuria=3.8g/24 hours

2010 July 6: Creatinine=2.16

2010 July 29: Genetic testing shows Fibrinogen Glu526Val mutation.

2010 October 27: Creatinine=2.45 2010 November: Not accepted as candidate for kidney transplant. Kidney function too high.

2011 June 7: Creatinine=2.78; GFR=17
2011 July: Kidney infection. Creatinine briefly at 4.6 on July 17. Down to 3.5 two days later. 2011 August 18: Creatinine=2.86; GFR=16
2011 September 1: Creatinine=2.45; GFR=19
2011 December 1: Creatinine=3.38; GFR=13

2012 January: Not accepted as candidate for liver-only transplant. Kidney function too low.

2012 February 22: Creatinine=4.20; GFR=10; Protein in 24-hour urine=5254 mg
2012 April 19: Creatinine=3.76; GFR=12
2012 May 17: Creatinine=3.87; GFR=11

2012 June 28: Surgery for insertion of peritoneal dialysis catheter.

2012 July 24-26: Started training for peritoneal dialysis.

2012 July 31: Surgery on right side of chest cavity. Talc pleurodesis and diaphragm sealing.

2012 August 28: Approved for kidney transplant if a living donor can be found.

2012 October 2-8: Resumed training for peritoneal dialysis. Creatinine=7.5

2012 October 9: Hospitalized to start hemodialysis.
2012 October 10: Permacath inserted in chest. First hemodialysis treatment.
2012 October 12: Second hemodialysis treatment. AV fistula created in right elbow. Catheter for peritoneal dialysis removed.
2012 October 15: Released from hospital after third hemodialysis treatment.

2012 October 26: Permacath replaced due to excessive bleeding at entry site.

2012 November 8: Stress test and cardiologist visit. No heart abnormalities noted.
2012 November 16: Determined to be allergic to heparin (anticoagulant). Since she also cannot take Coumadin, decision was made not to use an anticoagulant during dialysis.

2012 December 6: Permacath replaced due to infection.
2012 December 13 and 15: First use of fistula for dialysis access. Fistula use halted temporarily due to severe bleeding under the skin.

2013 January 7: Resumed use of fistula for dialysis access.
2013 January 25: Length of dialysis sessions increased from 3.5 to 4.0 hours.

2013 February 8: Permacath removed due to infection.
2013 February 26: Balloon angioplasty on right arm.

2013 March 14: Balloon angioplasty on right arm.
2013 March 15: Permacath inserted on left side due to issues with fistula access in right arm.

2013 April 1: A piece of surgical felt purposely left inside when PD catheter was removed (October 2012) is causing irritation.
2013 April 4: Permacath removed.
2013 April 16: Needle biopsy for lump on left breast. Benign.
2013 April 30: Creatinine=4.56

2013 June 8: Emergency room visit due to infection at site of PD catheter removal.
2013 June 11: Day surgery to remove surgical felt at site of PD catheter removal.
2013 June 28: Emergency dialysis session on cruise ship to remove excess fluid.

2013 July 3: Emergency surgery for infection at site of PD catheter removal.
2013 July 5: Released from hospital with wound vacuum for surgery site. Wound vacuum in place until July 19.
2013 July 25: Mohs surgery for skin cancer on right leg.

2013 September: Creatinine=6.63

2013 August 16: Heart catheterization. No abnormalities found.
2013 August 30: Recent bloodwork shows high calcium level. Parathyroid ruled out as cause.

2013 October 1: Bone scan to determine if bone cancer is cause of high calcium. No abnormalities found.

2013 November 1: Removed from kidney transplant waiting list.
2013 November 8: New blood pressure medications added. Blood pressure becoming more difficult to control.
2013 November 12: CT scan of chest, abdomen and pelvis. Only abnormality noted was in area of talc pleurodesis surgery.

2014 May: Back on kidney transplant waiting list.
2014 June 10: Creatinine=5.49

2015 March: Kidney transplant on hold due to medical issues. (GI bleed and pulmonary nodules)

2015 June 23: Creatinine=4.94

2015 October 17: Died due to apparent rupture of AV fistula. (FVAH: Fatal Vascular Access Hemorrhage)
(Individual last updated December 16, 2017.)

David J. (DOB July 1962) (Son of Linda J.)

(No offspring)
(Two siblings: One negative, one untested.)
(Added to page November 17, 2012)

2010 October: No proteinuria. Creatinine level normal.

2011 February: Genetic testing shows Fibrinogen Glu526Val mutation.

2011 December: No proteinuria (including 24-hour urine collection). Creatinine =1.0 

2013 January: No proteinuria. Creatinine=1.13, GFR=75

2014 January: No proteinuria. Creatinine=1.0. GFR>59

2015 February: No proteinuria. Creatinine=1.0. GFR=78

2016 February: No proteinuria. Creatinine=1.0. GFR=77
Mild anemia. Hemoglobin=12.0

2016 March: Hemoglobin=12.6. Ferritin=6
2016 August: Hemoglobin=13.3. Ferritin=5

2016 September: Started taking iron supplement
2016 October: Hemoglobin=14.3. Ferritin=15

2016 December: Hemoglobin=15.3. Ferritin=28

2017 January 1: Stopped taking iron supplement

2017 February: Hemoglobin=15.9. Ferritin=41.7. Creatinine=1.1. GFR=70.

2017 August: Hemoglobin=16.1. Creatinine=1.03. GFR>59.
(Individual last updated August 17, 2017.)

Marshall M. (February 1910 - October 1986) (obligate carrier) (Son of Selma and Arrie M.)
(Three offspring: One positive (Linda J.), two untested.)
(8 adult siblings, 7 with offspring)
(Added to page July 5, 2013)

No known kidney issues at time of death.
(Individual last updated January 7, 2014.)

Charlie M. (February 1926 - January 1987) (obligate carrier) (Son of Selma and Arrie M.)
(Three offspring: One positive (RM), two negative.)
(8 adult siblings, 7 with offspring)
(Added to page July 5, 2013)

No known kidney issues at time of death.
(Individual last updated January 7, 2014.)

RM (Son of Charlie M.)
(Two offspring: Both untested.)
(2 siblings, both negative)
(Added to page July 5, 2013)

~2011: On hemodialysis.
(Individual last updated August 1, 2013.)

Kindred #2
(Added to page October 30, 2013)

Cathy T. (DOB March 1951)
(Five offspring: One tested negative as of 2017.)
(Added to page October 30, 2013)

2009 September: Diagnosed with amyloidosis, type unknown. Creatinine=1.77
2009 October: Creatinine=1.64; GFR=32
                          Diagnosed with fibrinogen amyloidosis after kidney biopsy analyzed by mass spec at Mayo Clinic.
                          Creatinine=2.05; GFR=25

2009 November: Creatinine=1.80; GFR=29. Approved for liver transplant at UCSF.

2010 February: Creatinine=1.97; GFR=26
2010 March: Approved for domino liver transplant.

2010 June: Kidney function around 23%. Anemia getting worse.
2010 July 12: Received liver transplant at UCSF. (Middle patient of domino transplant.)
2010 July 19: Kidney function at January 2010 levels.

2010 August 15: Kidney function at 11%.
2010 September 28: Kidney function at 28%.

2010 October 7: Removed from kidney transplant waiting list due to improved kidney function.
2010 November 16: Mild to moderate rejection of transplanted liver. Treated with prednisone.

2011 December: Creatinine=2.2; GFR=24

2014 February: Creatinine=1.9; GFR=27

2015 January: Creatinine=1.7; GFR=30

2016 February: Creatinine=1.8
(Individual last updated November 10, 2017.)

Kindred #3

(This could be you. Just let me know . . . )

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